Sample records for tansey william gene

  1. Intracranial arteries in individuals with the elastin gene hemideletion of Williams syndrome.

    PubMed

    Wint, D P; Butman, J A; Masdeu, J C; Meyer-Lindenberg, A; Mervis, C B; Sarpal, D; Morris, C A; Berman, K F

    2014-01-01

    Williams syndrome, a rare genetic disorder with a striking neurobehavioral profile characterized by extreme sociability and impaired visuospatial construction abilities, is caused by a hemideletion that includes the elastin gene, resulting in frequent supravavular aortic stenosis and other stenotic arterial lesions. Strokes have been reported in Williams syndrome. Although the extracranial carotid artery has been studied in a sample of patients with Williams syndrome, proximal intracranial arteries have not. Using MRA, we studied the intracranial vessels in 27 participants: 14 patients with Williams syndrome (age range, 18-44 years; mean age, 27.3 ± 9.1; 43% women) and 13 healthy control participants with similar age and sex distribution (age range, 22-52 years; mean age, 33.4 ± 7.6; 46% women). All participants with Williams syndrome had hemideletions of the elastin gene. Blinded to group allocation or to any other clinical data, a neuroradiologist determined the presence of intracranial vascular changes in the 2 groups. The Williams syndrome group and the healthy control group had similar patency of the proximal intracranial arteries, including the internal carotid and vertebral arteries; basilar artery; and stem and proximal branches of the anterior cerebral artery, MCA, and posterior cerebral arteries. The postcommunicating segment of the anterior cerebral artery was longer in the Williams syndrome group. Despite the elastin haploinsufficiency, the proximal intracranial arteries in Williams syndrome preserve normal patency.

  2. Cognitive-behavioral phenotypes of Williams syndrome are associated with genetic variation in the GTF2I gene, in a healthy population.

    PubMed

    Crespi, Bernard J; Hurd, Peter L

    2014-11-28

    Individuals with Williams syndrome, a neurogenetic condition caused by deletion of a set of genes at chromosomal location 7q11.23, exhibit a remarkable suite of traits including hypersociality with high, nonselective friendliness and low social anxiety, expressive language relatively well-developed but under-developed social-communication skills overall, and reduced visual-spatial abilities. Deletions and duplications of the Williams-syndrome region have also been associated with autism, and with schizophrenia, two disorders centrally involving social cognition. Several lines of evidence have linked the gene GTF2I (General Transcription Factor IIi) with the social phenotypes of Williams syndrome, but a role for this gene in sociality within healthy populations has yet to be investigated. We genotyped a large set of healthy individuals for two single-nucleotide polymorphisms in the GTF2I gene that have recently been significantly associated with autism, and thus apparently exhibit functional effects on autism-related social phenotypes. GTF2I genotypes for these SNPs showed highly significant association with low social anxiety combined with reduced social-communication abilities, which represents a metric of the Williams-syndrome cognitive profile as described from previous studies. These findings implicate the GTF2I gene in the neurogenetic basis of social communication and social anxiety, both in Williams syndrome and among individuals in healthy populations.

  3. A human neurodevelopmental model for Williams syndrome.

    PubMed

    Chailangkarn, Thanathom; Trujillo, Cleber A; Freitas, Beatriz C; Hrvoj-Mihic, Branka; Herai, Roberto H; Yu, Diana X; Brown, Timothy T; Marchetto, Maria C; Bardy, Cedric; McHenry, Lauren; Stefanacci, Lisa; Järvinen, Anna; Searcy, Yvonne M; DeWitt, Michelle; Wong, Wenny; Lai, Philip; Ard, M Colin; Hanson, Kari L; Romero, Sarah; Jacobs, Bob; Dale, Anders M; Dai, Li; Korenberg, Julie R; Gage, Fred H; Bellugi, Ursula; Halgren, Eric; Semendeferi, Katerina; Muotri, Alysson R

    2016-08-18

    Williams syndrome is a genetic neurodevelopmental disorder characterized by an uncommon hypersociability and a mosaic of retained and compromised linguistic and cognitive abilities. Nearly all clinically diagnosed individuals with Williams syndrome lack precisely the same set of genes, with breakpoints in chromosome band 7q11.23 (refs 1-5). The contribution of specific genes to the neuroanatomical and functional alterations, leading to behavioural pathologies in humans, remains largely unexplored. Here we investigate neural progenitor cells and cortical neurons derived from Williams syndrome and typically developing induced pluripotent stem cells. Neural progenitor cells in Williams syndrome have an increased doubling time and apoptosis compared with typically developing neural progenitor cells. Using an individual with atypical Williams syndrome, we narrowed this cellular phenotype to a single gene candidate, frizzled 9 (FZD9). At the neuronal stage, layer V/VI cortical neurons derived from Williams syndrome were characterized by longer total dendrites, increased numbers of spines and synapses, aberrant calcium oscillation and altered network connectivity. Morphometric alterations observed in neurons from Williams syndrome were validated after Golgi staining of post-mortem layer V/VI cortical neurons. This model of human induced pluripotent stem cells fills the current knowledge gap in the cellular biology of Williams syndrome and could lead to further insights into the molecular mechanism underlying the disorder and the human social brain.

  4. Genes, language, and the nature of scientific explanations: the case of Williams syndrome.

    PubMed

    Musolino, Julien; Landau, Barbara

    2012-01-01

    In this article, we discuss two experiments of nature and their implications for the sciences of the mind. The first, Williams syndrome, bears on one of cognitive science's holy grails: the possibility of unravelling the causal chain between genes and cognition. We sketch the outline of a general framework to study the relationship between genes and cognition, focusing as our case study on the development of language in individuals with Williams syndrome. Our approach emphasizes the role of three key ingredients: the need to specify a clear level of analysis, the need to provide a theoretical account of the relevant cognitive structure at that level, and the importance of the (typical) developmental process itself. The promise offered by the case of Williams syndrome has also given rise to two strongly conflicting theoretical approaches-modularity and neuroconstructivism-themselves offshoots of a perennial debate between nativism and empiricism. We apply our framework to explore the tension created by these two conflicting perspectives. To this end, we discuss a second experiment of nature, which allows us to compare the two competing perspectives in what comes close to a controlled experimental setting. From this comparison, we conclude that the "meaningful debate assumption", a widespread assumption suggesting that neuroconstructivism and modularity address the same questions and represent genuine theoretical alternatives, rests on a fallacy.

  5. Hemizygosity at the elastin locus and clinical features of Williams syndrome

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Morimoto, Y; Kuwano, A.; Kuwajima, K.

    1994-09-01

    Williams syndrome is a recognizable syndrome characterized by distinctive facial appearance, gregarious personality, mental retardation, congenital heart defect, particularly supravalvular aortic stenosis (SVAS), and joint limitation. SVAS is an autosomal vascular disorder and the elastin gene was disrupted in patients with SVAS. Ewat et al. reported that hemizygosity at the elastin locus was detected in four familial and five sporadic cases of Williams syndrome. However, three patients did not have SVAS. We reconfirmed hemizygosity at the elastin locus in five patients with typical clinical features of Williams syndrome. Hemizygosity was detected in four cases with SVAS. However, one patient withmore » distinctive facial appearance and typical Williams syndrome personality had two alleles of the elastin gene, but he did not have the congenital heart anomaly. Williams syndrome is thought to be a contiguous gene disorder. Thus, our data suggest that the elastin gene is responsible for the vascular defect in patients with Williams syndrome, and flanking genes are responsible for characteristic facial appearance and personality.« less

  6. A new case of keratoconus associated with Williams-Beuren syndrome.

    PubMed

    Viana, Melissa Machado; Frasson, Maria; Leão, Letícia Lima; Stofanko, Martin; Gonçalves-Dornelas, Higgor; Cunha, Pricila da Silva; de Aguiar, Marcos José Burle

    2013-09-01

    Williams-Beuren syndrome is a multisystemic genetic disorder caused by a contiguous gene deletion at 7q11.23. Keratoconus is a complex disease and it is suspected to have a genetic origin, although the specific gene responsible for keratoconus has not been identified. Although there are several ocular features in Williams-Beuren syndrome, keratoconus is not regularly described as part of this syndrome. To report a new patient with keratoconus and Williams-Beuren syndrome. This is the third case of an association between Williams-Beuren syndrome and keratoconus. The authors believe that the Williams-Beuren syndrome chromosome region can be a possible target for further investigation as the genetic basis of keratoconus.

  7. Language and Communicative Development in Williams Syndrome

    ERIC Educational Resources Information Center

    Mervis, Carolyn B.; Becerra, Angela M.

    2007-01-01

    Williams syndrome, a genetic disorder caused by a microdeletion of approximately 25 genes on chromosome 7q11.23, is associated with mild to moderate intellectual disability or learning difficulties. Most individuals with Williams syndrome evidence a cognitive profile including relative strengths in verbal short-term memory and language, and…

  8. William's syndrome: gene expression is related to parental origin and regional coordinate control

    PubMed Central

    Collette, Jeremy C; Chen, Xiao-Ning; Mills, Debra L; Galaburda, Albert M; Reiss, Allan L; Bellugi, Ursula; Korenberg, Julie R

    2013-01-01

    William's syndrome (WS) features a spectrum of neurocognitive and behavioral abnormalities due to a rare 1.5MB deletion that includes about 24–28 genes on chromosome band 7q11.23. Study of the expression of these genes from the single normal copy provides an opportunity to elucidate the genetic and epigenetic controls on these genes as well as their roles in both WS and normal brain development and function. We used quantitative RT-PCR to determine the transcriptional level of 14 WS gene markers in a cohort of 77 persons with WS and 48 normal controls. Results reported here: (1) show that the expression of the genes deleted in WS is decreased in some but not all cases, (2) demonstrate that the parental origin of the deletion contributes to the level of expression of GTF2I independently of age and gender and (3) indicate that the correlation of expression between GTF2I and some other genes in the WS region differs in WS subjects and normal controls, which in turn points toward a regulatory role for this gene. Interspecies comparisons suggest GTF2I may play a key role in normal brain development. PMID:19282872

  9. The contribution of CLIP2 haploinsufficiency to the clinical manifestations of the Williams-Beuren syndrome.

    PubMed

    Vandeweyer, Geert; Van der Aa, Nathalie; Reyniers, Edwin; Kooy, R Frank

    2012-06-08

    Williams-Beuren syndrome is a rare contiguous gene syndrome, characterized by intellectual disability, facial dysmorphisms, connective-tissue abnormalities, cardiac defects, structural brain abnormalities, and transient infantile hypercalcemia. Genes lying telomeric to RFC2, including CLIP2, GTF2I and GTF2IRD1, are currently thought to be the most likely major contributors to the typical Williams syndrome cognitive profile, characterized by a better-than-expected auditory rote-memory ability, a relative sparing of language capabilities, and a severe visual-spatial constructive impairment. Atypical deletions in the region have helped to establish genotype-phenotype correlations. So far, however, hardly any deletions affecting only a single gene in the disease region have been described. We present here two healthy siblings with a pure, hemizygous deletion of CLIP2. A putative role in the cognitive and behavioral abnormalities seen in Williams-Beuren patients has been suggested for this gene on the basis of observations in a knock-out mouse model. The presented siblings did not show any of the clinical features associated with the syndrome. Cognitive testing showed an average IQ for both and no indication of the Williams syndrome cognitive profile. This shows that CLIP2 haploinsufficiency by itself does not lead to the physical or cognitive characteristics of the Williams-Beuren syndrome, nor does it lead to the Williams syndrome cognitive profile. Although contribution of CLIP2 to the phenotype cannot be excluded when it is deleted in combination with other genes, our results support the hypothesis that GTF2IRD1 and GTF2I are the main genes causing the cognitive defects associated with Williams-Beuren syndrome. Copyright © 2012 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  10. Nature and Nurture: Williams Syndrome across Cultures

    ERIC Educational Resources Information Center

    Zitzer-Comfort, Carol; Doyle, Teresa; Masataka, Nobuo; Korenberg, Julie; Bellugi, Ursula

    2007-01-01

    This study is concerned with ways in which children with Williams syndrome (WS), a rare neurodevelopmental disorder arising from a hemizygous deletion in chromosome band 7q11.23 including the gene for elastin (ELN) and approximately 20 surrounding genes, are affected by social mores of vastly differing cultures: the United States and Japan. WS…

  11. Williams syndrome

    MedlinePlus

    ... with Williams syndrome may show: A flattened nasal bridge with small upturned nose Long ridges in the ... Alternative Names Williams-Beuren syndrome Images Low nasal bridge Chromosomes and DNA References Morris CA. Williams syndrome. ...

  12. The Williams syndrome prosociality gene GTF2I mediates oxytocin reactivity and social anxiety in a healthy population.

    PubMed

    Procyshyn, Tanya L; Spence, Jason; Read, Silven; Watson, Neil V; Crespi, Bernard J

    2017-04-01

    The neurohormone oxytocin plays a central role in human social behaviour and cognition, and oxytocin dysregulation may contribute to psychiatric disorders. However, genetic factors influencing individual variation in the oxytocinergic system remain poorly understood. We genotyped 169 healthy adults for a functional polymorphism in GTF2I ( general transcription factor II-I ), a gene associated with high prosociality and reduced social anxiety in Williams syndrome, a condition reported to involve high oxytocin levels and reactivity. Participants' salivary oxytocin levels were measured before and after watching a validated empathy-inducing video. Oxytocin reactivity, defined as pre- to post-video percentage change in salivary oxytocin, varied substantially and significantly between individuals with different GTF2I genotypes, with, additionally, a trend towards an interaction between genotype and sex. Individuals with more oxytocin-reactive genotypes also reported significantly lower social anxiety. These findings suggest a model whereby GTF2I has a continuum of effects on human sociality, from the extreme social phenotypes and oxytocin dysregulation associated with gene deletion in Williams syndrome, to individual differences in oxytocin reactivity and sociality associated with common polymorphisms in healthy populations. © 2017 The Author(s).

  13. Congenital heart defects in Williams syndrome.

    PubMed

    Yuan, Shi-Min

    2017-01-01

    Yuan SM. Congenital heart defects in Williams syndrome. Turk J Pediatr 2017; 59: 225-232. Williams syndrome (WS), also known as Williams-Beuren syndrome, is a rare genetic disorder involving multiple systems including the circulatory system. However, the etiologies of the associated congenital heart defects in WS patients have not been sufficiently elucidated and represent therapeutic challenges. The typical congenital heart defects in WS were supravalvar aortic stenosis, pulmonary stenosis (both valvular and peripheral), aortic coarctation and mitral valvar prolapse. The atypical cardiovascular anomalies include tetralogy of Fallot, atrial septal defects, aortic and mitral valvular insufficiencies, bicuspid aortic valves, ventricular septal defects, total anomalous pulmonary venous return, double chambered right ventricle, Ebstein anomaly and arterial anomalies. Deletion of the elastin gene on chromosome 7q11.23 leads to deficiency or abnormal deposition of elastin during cardiovascular development, thereby leading to widespread cardiovascular abnormalities in WS. In this article, the distribution, treatment and surgical outcomes of typical and atypical cardiac defects in WS are discussed.

  14. Molecular basis of Williams-Beuren syndrome: TFII-I regulated targets involved in craniofacial development.

    PubMed

    Makeyev, Aleksandr V; Bayarsaihan, Dashzeveg

    2011-01-01

    The aim of this study is to identify gene targets of TFII-I transcription factors involved in craniofacial development. Recent findings in individuals with Williams-Beuren syndrome who show facial dysmorphism and cognitive defects have pointed to TFII-I genes (GTF2I and GTF2IRD1) as the prime candidates responsible for these clinical features. However, TFII-I proteins are multifunctional transcriptional factors regulating a number of genes during development, and how their haploinsufficiency leads to the Williams-Beuren syndrome phenotype is currently unknown. Here we report the identification of three genes with a well-established relevance to craniofacial development as direct TFII-I targets. These genes, craniofacial development protein 1 (Cfdp1), Sec23 homolog A (Sec23a), and nuclear receptor binding SET domain protein 1 (Nsd1), contain consensus TFII-I binding sites in their proximal promoters; the chromatin immunoprecipitation analysis showed that TFII-I transcription factors are recruited to these sites in vivo. The results suggest that transcriptional regulation of these genes by TFII-I proteins could provide a possible genotype-phenotype link in Williams-Beuren syndrome.

  15. Dental management of patient with Williams Syndrome - A case report.

    PubMed

    Wong, Daniel; Ramachandra, Srinivas Sulugodu; Singh, Ashish Kumar

    2015-01-01

    Williams syndrome is a multisystemic rare genetic disorder caused by deletion of 26-28 genes in the long arm of chromosome 7. It is characterized by developmental and physical abnormalities including congenital cardiovascular abnormalities, mental retardation, neurological features, growth deficiency, genitourinary manifestations, gastrointestinal problems, musculoskeletal problems, unique behavioral characteristics, and dental problems. Dental abnormalities include malocclusion, hypodontia, malformed teeth, taurodontism, pulp stones, increased space between teeth, enamel hypoplasia, and high prevalence of dental caries. Authors report a 17-year-old female patient with underlying Williams syndrome. Oral features and problems seen in the patient are listed. Malocclusion and screwdriver shaped teeth were noticed. Generalized widening of the periodontal ligament space with vital teeth was seen. This finding has not been reported in cases of Williams syndrome earlier. Precautions taken during dental treatment in patients with Williams syndrome are also discussed.

  16. Williams Syndrome and 15q Duplication: Coincidence versus Association.

    PubMed

    Khokhar, Aditi; Agarwal, Swashti; Perez-Colon, Sheila

    2017-01-01

    Williams syndrome is a multisystem disorder caused by contiguous gene deletion in 7q11.23, commonly associated with distinctive facial features, supravalvular aortic stenosis, short stature, idiopathic hypercalcemia, developmental delay, joint laxity, and a friendly personality. The clinical features of 15q11q13 duplication syndrome include autism, mental retardation, ataxia, seizures, developmental delay, and behavioral problems. We report a rare case of a girl with genetically confirmed Williams syndrome and coexisting 15q duplication syndrome. The patient underwent treatment for central precocious puberty and later presented with primary amenorrhea. The karyotype revealed 47,XX,+mar. FISH analysis for the marker chromosome showed partial trisomy/tetrasomy for proximal chromosome 15q (15p13q13). FISH using an ELN -specific probe demonstrated a deletion in the Williams syndrome critical region in 7q11.23. To our knowledge, a coexistence of Williams syndrome and 15q duplication syndrome has not been reported in the literature. Our patient had early pubertal development, which has been described in some patients with Williams syndrome. However, years later after discontinuing gonadotropin-releasing hormone analogue treatment, she developed primary amenorrhea.

  17. Prevalence of scoliosis in Williams-Beuren syndrome patients treated at a regional reference center

    PubMed Central

    Damasceno, Marcelo Loquette; Cristante, Alexandre Fogaça; Marcon, Raphael Martus; de Barros Filho, Tarcísio Eloy Pessoa

    2014-01-01

    OBJECTIVE: This study assessed the prevalence of scoliosis and the patterns of scoliotic curves in patients with Williams-Beuren syndrome. Williams-Beuren syndrome is caused by a chromosome 7q11.23 deletion in a region containing 28 genes, with the gene encoding elastin situated approximately at the midpoint of the deletion. Mutation of the elastin gene leads to phenotypic changes in patients, including neurodevelopmental impairment of varying degrees, characteristic facies, cardiovascular abnormalities, hypercalcemia, urological dysfunctions, and bone and joint dysfunctions. METHODS: A total of 41 patients diagnosed with Williams-Beuren syndrome, who were followed up at the genetics ambulatory center of a large referral hospital, were included in the study. There were 25 male subjects. The patients were examined and submitted to radiographic investigation for Cobb angle calculation. RESULTS: It was observed that 14 patients had scoliosis; of these 14 patients, 10 were male. The pattern of deformity in younger patients was that of flexible and simple curves, although adults presented with double and triple curves. Statistical analysis showed no relationships between scoliosis and age or sex. CONCLUSION: This study revealed a prevalence of scoliosis in patients with Williams-Beuren syndrome of 34.1%; however, age and sex were not significantly associated with scoliosis or with the severity of the curves. PMID:25029575

  18. [Williams-Beuren syndrome (Williams syndrome). Case report].

    PubMed

    Miklós, Györgyi; Fekete, György; Haltrich, Irén; Tóth, Miklós; Reismann, Péter

    2017-11-01

    Williams syndrome is a rare genetic disorder, that occurs equally in all ethnic groups and both sexes. The diagnosis might be missed during childhood in mild cases. However, establishing the diagnosis is important, not only to find the cause of intellectual disability but to look for cardiovascular, endocrine, psychiatry, urology and other conditions, which can occur at any age in the patients' lifetime. This case report presents the story of 47-year-old woman, who was admitted with haematemesis. During her stay on the ward, in the light of the distinctive facial features, mental retardation, and social behaviour patterns, the possibility of Williams syndrome emerged. Later, the diagnosis was confirmed by genetic analysis. This female is the oldest living patient with Williams syndrome in Hungary. Orv Hetil. 2017; 158(47): 1883-1888.

  19. 33 CFR 167.1702 - In Prince William Sound: Prince William Sound Traffic Separation Scheme.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... William Sound Traffic Separation Scheme. 167.1702 Section 167.1702 Navigation and Navigable Waters COAST... SEPARATION SCHEMES Description of Traffic Separation Schemes and Precautionary Areas Pacific West Coast § 167.1702 In Prince William Sound: Prince William Sound Traffic Separation Scheme. The Prince William Sound...

  20. 33 CFR 167.1702 - In Prince William Sound: Prince William Sound Traffic Separation Scheme.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... William Sound Traffic Separation Scheme. 167.1702 Section 167.1702 Navigation and Navigable Waters COAST... SEPARATION SCHEMES Description of Traffic Separation Schemes and Precautionary Areas Pacific West Coast § 167.1702 In Prince William Sound: Prince William Sound Traffic Separation Scheme. The Prince William Sound...

  1. 33 CFR 167.1702 - In Prince William Sound: Prince William Sound Traffic Separation Scheme.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... William Sound Traffic Separation Scheme. 167.1702 Section 167.1702 Navigation and Navigable Waters COAST... SEPARATION SCHEMES Description of Traffic Separation Schemes and Precautionary Areas Pacific West Coast § 167.1702 In Prince William Sound: Prince William Sound Traffic Separation Scheme. The Prince William Sound...

  2. 33 CFR 167.1702 - In Prince William Sound: Prince William Sound Traffic Separation Scheme.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... William Sound Traffic Separation Scheme. 167.1702 Section 167.1702 Navigation and Navigable Waters COAST... SEPARATION SCHEMES Description of Traffic Separation Schemes and Precautionary Areas Pacific West Coast § 167.1702 In Prince William Sound: Prince William Sound Traffic Separation Scheme. The Prince William Sound...

  3. 33 CFR 167.1702 - In Prince William Sound: Prince William Sound Traffic Separation Scheme.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... William Sound Traffic Separation Scheme. 167.1702 Section 167.1702 Navigation and Navigable Waters COAST... SEPARATION SCHEMES Description of Traffic Separation Schemes and Precautionary Areas Pacific West Coast § 167.1702 In Prince William Sound: Prince William Sound Traffic Separation Scheme. The Prince William Sound...

  4. Williams syndrome deletions and duplications: Genetic windows to understanding anxiety, sociality, autism, and schizophrenia.

    PubMed

    Crespi, Bernard J; Procyshyn, Tanya L

    2017-08-01

    We describe and evaluate an integrative hypothesis for helping to explain the major neurocognitive features of individuals with Williams syndrome region deletions and duplications. First, we demonstrate how the cognitive differences between Williams syndrome individuals, individuals with duplications of this region, and healthy individuals parallel the differences between individuals subject to effects of increased or decreased oxytocin. Second, we synthesize evidence showing that variation in expression of the gene GTF2I (General Transcription Factor II-I) underlies the primary social phenotypes of Williams syndrome and that common genetic variation in GTF2I mediates oxytocin reactivity, and its correlates, in healthy populations. Third, we describe findings relevant to the hypothesis that the GTF2I gene is subject to parent of origin effects whose behavioral expression fits with predictions from the kinship theory of genomic imprinting. Fourth, we describe how Williams syndrome can be considered, in part, as an autistic syndrome of Lorna Wing's 'active-but-odd' autism subtype, in contrast to associations of duplications with both schizophrenia and autism. Copyright © 2017 Elsevier Ltd. All rights reserved.

  5. Williams syndrome predisposes to vascular stiffness modified by antihypertensive use and copy number changes in NCF1.

    PubMed

    Kozel, Beth A; Danback, Joshua R; Waxler, Jessica L; Knutsen, Russell H; de Las Fuentes, Lisa; Reusz, Gyorgy S; Kis, Eva; Bhatt, Ami B; Pober, Barbara R

    2014-01-01

    Williams syndrome is caused by the deletion of 26 to 28 genes, including elastin, on human chromosome 7. Elastin insufficiency leads to the cardiovascular hallmarks of this condition, namely focal stenosis and hypertension. Extrapolation from the Eln(+/-) mouse suggests that affected people may also have stiff vasculature, a risk factor for stroke, myocardial infarction, and cardiac death. NCF1, one of the variably deleted Williams genes, is a component of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex and is involved in the generation of oxidative stress, making it an interesting candidate modifier for vascular stiffness. Using a case-control design, vascular stiffness was evaluated by pulse wave velocity in 77 Williams cases and matched controls. Cases had stiffer conducting vessels than controls (P<0.001), with increased stiffness observed in even the youngest children with Williams syndrome. Pulse wave velocity increased with age at comparable rates in cases and controls, and although the degree of vascular stiffness varied, it was seen in both hypertensive and normotensive Williams participants. Use of antihypertensive medication and extension of the Williams deletion to include NCF1 were associated with protection from vascular stiffness. These findings demonstrate that vascular stiffness is a primary vascular phenotype in Williams syndrome and that treatment with antihypertensives or agents inhibiting oxidative stress may be important in managing patients with this condition, potentially even those who are not overtly hypertensive.

  6. Variation in the Williams syndrome GTF2I gene and anxiety proneness interactively affect prefrontal cortical response to aversive stimuli.

    PubMed

    Jabbi, M; Chen, Q; Turner, N; Kohn, P; White, M; Kippenhan, J S; Dickinson, D; Kolachana, B; Mattay, V; Weinberger, D R; Berman, K F

    2015-08-18

    Characterizing the molecular mechanisms underlying the heritability of complex behavioral traits such as human anxiety remains a challenging endeavor for behavioral neuroscience. Copy-number variation (CNV) in the general transcription factor gene, GTF2I, located in the 7q11.23 chromosomal region that is hemideleted in Williams syndrome and duplicated in the 7q11.23 duplication syndrome (Dup7), is associated with gene-dose-dependent anxiety in mouse models and in both Williams syndrome and Dup7. Because of this recent preclinical and clinical identification of a genetic influence on anxiety, we examined whether sequence variation in GTF2I, specifically the single-nucleotide polymorphism rs2527367, interacts with trait and state anxiety to collectively impact neural response to anxiety-laden social stimuli. Two hundred and sixty healthy adults completed the Tridimensional Personality Questionnaire Harm Avoidance (HA) subscale, a trait measure of anxiety proneness, and underwent functional magnetic resonance imaging (fMRI) while matching aversive (fearful or angry) facial identity. We found an interaction between GTF2I allelic variations and HA that affects brain response: in individuals homozygous for the major allele, there was no correlation between HA and whole-brain response to aversive cues, whereas in heterozygotes and individuals homozygous for the minor allele, there was a positive correlation between HA sub-scores and a selective dorsolateral prefrontal cortex (DLPFC) responsivity during the processing of aversive stimuli. These results demonstrate that sequence variation in the GTF2I gene influences the relationship between trait anxiety and brain response to aversive social cues in healthy individuals, supporting a role for this neurogenetic mechanism in anxiety.

  7. Williams Syndrome: A Relationship between Genetics, Brain Morphology and Behaviour

    ERIC Educational Resources Information Center

    Fahim, C.; Yoon, U.; Nashaat, N. H.; Khalil, A. K.; El-Belbesy, M.; Mancini-Marie, A.; Evans, A. C.; Meguid, N.

    2012-01-01

    Background: Genetically Williams syndrome (WS) promises to provide essential insight into the pathophysiology of cortical development because its ~28 deleted genes are crucial for cortical neuronal migration and maturation. Phenotypically, WS is one of the most puzzling childhood neurodevelopmental disorders affecting most intellectual…

  8. Language and Literacy Development of Children with Williams Syndrome

    ERIC Educational Resources Information Center

    Mervis, Carolyn B.

    2009-01-01

    Williams syndrome is a rare neurodevelopmental disorder caused by deletion of approximately 25 genes on chromosome 7q11.23. Children with the syndrome evidence large individual differences in both broad language and reading abilities. Nevertheless, as a group, children with this syndrome show a consistent pattern characterized by relative…

  9. Conceptualizing neurodevelopmental disorders through a mechanistic understanding of fragile X syndrome and Williams syndrome.

    PubMed

    Fung, Lawrence K; Quintin, Eve-Marie; Haas, Brian W; Reiss, Allan L

    2012-04-01

    The overarching goal of this review is to compare and contrast the cognitive-behavioral features of fragile X syndrome (FraX) and Williams syndrome and to review the putative neural and molecular underpinnings of these features. Information is presented in a framework that provides guiding principles for conceptualizing gene-brain-behavior associations in neurodevelopmental disorders. Abnormalities, in particular cognitive-behavioral domains with similarities in underlying neurodevelopmental correlates, occur in both FraX and Williams syndrome including aberrant frontostriatal pathways leading to executive function deficits, and magnocellular/dorsal visual stream, superior parietal lobe, inferior parietal lobe, and postcentral gyrus abnormalities contributing to deficits in visuospatial function. Compelling cognitive-behavioral and neurodevelopmental contrasts also exist in these two disorders, for example, aberrant amygdala and fusiform cortex structure and function occurring in the context of contrasting social behavioral phenotypes, and temporal cortical and cerebellar abnormalities potentially underlying differences in language function. Abnormal dendritic development is a shared neurodevelopmental morphologic feature between FraX and Williams syndrome. Commonalities in molecular machinery and processes across FraX and Williams syndrome occur as well - microRNAs involved in translational regulation of major synaptic proteins; scaffolding proteins in excitatory synapses; and proteins involved in axonal development. Although the genetic variations leading to FraX and Williams syndrome are different, important similarities and contrasts in the phenotype, neurocircuitry, molecular machinery, and cellular processes in these two disorders allow for a unique approach to conceptualizing gene-brain-behavior links occurring in neurodevelopmental disorders.

  10. Coronary ostium occlusion by coronary cusp displacement in Williams syndrome.

    PubMed

    Shiohama, Tadashi; Fujii, Katsunori; Ebata, Ryota; Funabashi, Nobusada; Matsumiya, Goro; Saito, Yuko Kazato; Takechi, Fumie; Yonemori, Yoko; Nakatani, Yukio; Shimojo, Naoki

    2016-06-01

    Williams syndrome is a contiguous gene deletion syndrome resulting from a heterozygous deletion on chromosome 7q11.23, and is characterized by distinctive facial features and supravalvular aortic stenosis (SVAS). This syndrome rarely presents unpredictable cardiac death, and yet, as illustrated in the present case, it is still not possible to predict it, even on close monitoring. We herein describe the case of a 6-year-old Japanese girl with Williams syndrome, who had sudden cardiac collapse due to cardiac infarction after pharyngitis. Cardiac failure followed a critical course that did not respond to catecholamine support or heart rest with extracardiac mechanical support. Although marked coronary stenosis was not present, the left coronary cusp abnormally adhered to the aortic wall, which may synergistically cause coronary ostium occlusion with SVAS. Altered hemodynamic state, even that caused by the common cold, may lead to critical myocardial events in Williams syndrome with SVAS. © 2015 Japan Pediatric Society.

  11. William Hunter and lymphatics.

    PubMed

    McDonald, Stuart W; Russell, David

    2018-04-03

    William Hunter along with his brother, John, and their colleagues William Hewson, William Cruikshank and John Sheldon made a large contribution to understanding of lymphatic vessels. Hewson, Cruikshank and Sheldon all carried out mercury injections and made much progress in mapping the distribution of lymphatics in the human body. William Hunter appreciated that lymphatics absorbed fluid from the tissues of the body and that lacteals of the intestine and lymphatics are similar structures. John Hunter carried out an elegant series of experiments that proved that lacteals absorb products of digestion. The Hunters, however, were wrong in dismissing absorption by blood vessels and missed the importance of blood capillaries. William Hewson showed that lymphatics were not confined to mammals but that they are present in reptiles, birds and fish. Hewson also demonstrated that tracheobronchial glands are lymph nodes and not mucus-secreting glands as previously thought. Although William Hunter appreciated that tuberculosis and venereal diseases might involve the regional lymph nodes, he does not seem to have fully grasped that malignant disease might involve the local nodes or the concept that knowledge of lymph drainage could be used to define the likely site of a primary malignancy. Copyright © 2018. Published by Elsevier GmbH.

  12. Williams works on the payload APEX TAGES in the JPM during Expedition 22

    NASA Image and Video Library

    2009-12-15

    ISS022-E-011304 (15 Dec. 2009) --- NASA astronaut Jeffrey Williams, Expedition 22 commander, conducts a daily status check of the Advanced Plant Experiments on Orbit (APEX) experiment in the Kibo laboratory of the International Space Station. During each check, Williams looks for health and color of the plants, since the Cambium plants are removed from the Advanced Biological Research System (ABRS). When completed, the APEX-Cambium payload in conjunction with the NASA-sponsored Transgenic Arabidopsis Gene Expression System (TAGES) will determine the role of gravity in Cambium wood cell development and demonstrate non-destructive reporter gene technology and investigate spaceflight plant stress. APEX-Cambium provides NASA and the ISS community a permanent controlled environment capability to support growth of various organisms (i.e. whole plants).

  13. William and Mary's President Exits on His Own Terms

    ERIC Educational Resources Information Center

    Fain, Paul

    2008-01-01

    The president and governing board at the College of William and Mary have parted ways in an unusually public split with a deeply partisan undercurrent. Gene R. Nichol says that the Board of Visitors forced him out for defending free speech and diversity on the campus, and that he turned down a generous severance package to go quietly. Board…

  14. Williams installs CBEF Cell Mechanosensing Humidifier

    NASA Image and Video Library

    2016-04-01

    ISS047e032018 (04/01/2016) --- NASA astronaut Jeff Williams works to install the Cell Biology Experiment Facility (CBEF) Cell Mechanosensing Humidifier. Cell Mechanosensing is a Japan Aerospace Exploration Agency (JAXA) investigation that identifies gravity sensors in skeletal muscle cells to develop countermeasures to muscle atrophy, a key space health issue. Scientists believe that the lack of mechanical stress from gravity causes tension fluctuations in the plasma membrane of skeletal muscle cells which changes the expression of key proteins and genes, and allows muscles to atrophy.

  15. Variations of transcript profiles between sea otters Enhydra lutris from Prince William Sound, Alaska, and clinically normal reference otters

    USGS Publications Warehouse

    Miles, A. Keith; Bowen, Lizabeth; Ballachey, Brenda E.; Bodkin, James L.; Murray, M.; Estes, J.L.; Keister, Robin A.; Stott, J.L.

    2012-01-01

    Development of blood leukocyte gene transcript profiles has the potential to expand condition assessments beyond those currently available to evaluate wildlife health, including sea otters Enhydra lutris, both individually and as populations. The 10 genes targeted in our study represent multiple physiological systems that play a role in immuno-modulation, inflammation, cell protection, tumor suppression, cellular stress-response, xenobiotic metabolizing enzymes, and antioxidant enzymes. These genes can be modified by biological, physical, or anthropogenic impacts and consequently provide information on the general type of stressors present in a given environment. We compared gene transcript profiles of sea otters sampled in 2008 among areas within Prince William Sound impacted to varying degrees by the 1989 ‘Exxon Valdez’ oil spill with those of captive and wild reference sea otters. Profiles of sea otters from Prince William Sound showed elevated transcription in genes associated with tumor formation, cell death, organic exposure, inflammation, and viral exposure when compared to the reference sea otter group, indicating possible recent and chronic exposure to organic contaminants. Sea otters from historically designated oiled areas within Prince William Sound 19 yr after the oil spill had higher transcription of genes associated with tumor formation, cell death, heat shock, and inflammation than those from areas designated as less impacted by the spill.

  16. Williams syndrome and mature B-Leukemia: A random association?

    PubMed

    Decimi, Valentina; Fazio, Grazia; Dell'Acqua, Fabiola; Maitz, Silvia; Galbiati, Marta; Rizzari, Carmelo; Biondi, Andrea; Cazzaniga, Giovanni; Selicorni, Angelo

    2016-12-01

    Williams syndrome (WBS) is a rare neurodevelopmental disorder with specific phenotypic characteristics and cardiac abnormalities, but is not considered as a cancer predisposing condition. However, in rare cases, malignancies have been described in patients with WBS, with hematologic cancer (mainly Burkitt Lymphoma and Acute Lymphoblastic Leukemia) as the most represented. We report here the case of a boy with WS and B-NHL. This is the unique case within the large cohort of patients (n = 117) followed in our institution for long time (mean clinical follow-up, 13 years). We herewith propose that the BCL7B gene, located in the chromosomal region commonly deleted in Williams syndrome, could potentially have a role in this particular association. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  17. Essential functions of the Williams-Beuren syndrome-associated TFII-I genes in embryonic development.

    PubMed

    Enkhmandakh, Badam; Makeyev, Aleksandr V; Erdenechimeg, Lkhamsuren; Ruddle, Frank H; Chimge, Nyam-Osor; Tussie-Luna, Maria Isabel; Roy, Ananda L; Bayarsaihan, Dashzeveg

    2009-01-06

    GTF2I and GTF2IRD1 encoding the multifunctional transcription factors TFII-I and BEN are clustered at the 7q11.23 region hemizygously deleted in Williams-Beuren syndrome (WBS), a complex multisystemic neurodevelopmental disorder. Although the biochemical properties of TFII-I family transcription factors have been studied in depth, little is known about the specialized contributions of these factors in pathways required for proper embryonic development. Here, we show that homozygous loss of either Gtf2ird1 or Gtf2i function results in multiple phenotypic manifestations, including embryonic lethality; brain hemorrhage; and vasculogenic, craniofacial, and neural tube defects in mice. Further analyses suggest that embryonic lethality may be attributable to defects in yolk sac vasculogenesis and angiogenesis. Microarray data indicate that the Gtf2ird1 homozygous phenotype is mainly caused by an impairment of the genes involved in the TGFbetaRII/Alk1/Smad5 signal transduction pathway. The effect of Gtf2i inactivation on this pathway is less prominent, but downregulation of the endothelial growth factor receptor-2 gene, resulting in the deterioration of vascular signaling, most likely exacerbates the severity of the Gtf2i mutant phenotype. A subset of Gtf2ird1 and Gtf2i heterozygotes displayed microcephaly, retarded growth, and skeletal and craniofacial defects, therefore showing that haploinsufficiency of TFII-I proteins causes various developmental anomalies that are often associated with WBS.

  18. Macrophage Polarization and Utility of in Vivo Therapy with a Brain-Permeable Anti-TNF Agent in Models of Autism

    DTIC Science & Technology

    2017-10-01

    of Autism PRINCIPAL INVESTIGATOR: MariadeLourdes Tansey, PhD CONTRACTING ORGANIZATION: Emory University Atlanta, GA 30322-4250 REPORT DATE... Autism 5a. CONTRACT NUMBER 5b. GRANT NUMBER 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR(S) MariadeLourdes Tansey, PhD 5d. PROJECT NUMBER 5e. TASK...response in the gut and do not make IL-17 which signals at receptors in the brain to mediate the autism -like behavior deficits. For this reason, we

  19. Identification of the WBSCR9 gene, encoding a novel transcriptional regulator, in the Williams-Beuren syndrome deletion at 7q11.23.

    PubMed

    Peoples, R J; Cisco, M J; Kaplan, P; Francke, U

    1998-01-01

    We have identified a novel gene (WBSCR9) within the common Williams-Beuren syndrome (WBS) deletion by interspecies sequence conservation. The WBSCR9 gene encodes a roughly 7-kb transcript with an open reading frame of 1483 amino acids and a predicted protein product size of 170.8 kDa. WBSCR9 is comprised of at least 20 exons extending over 60 kb. The transcript is expressed ubiquitously throughout development and is subject to alternative splicing. Functional motifs identified by sequence homology searches include a bromodomain; a PHD, or C4HC3, finger; several putative nuclear localization signals; four nuclear receptor binding motifs; a polyglutamate stretch and two PEST sequences. Bromodomains, PHD motifs and nuclear receptor binding motifs are cardinal features of proteins that are involved in chromatin remodeling and modulation of transcription. Haploinsufficiency for WBSCR9 gene products may contribute to the complex phenotype of WBS by interacting with tissue-specific regulatory factors during development.

  20. Conversations with John Williams

    ERIC Educational Resources Information Center

    Sullivan, Jack

    2007-01-01

    In this article, the author shares the views of John Williams, Hollywood's premier composer, who has written more than 300 scores, about the future of classical and film music. A gregarious person in a field requiring monklike isolation, Williams values the "association with the soloists, and the wonderful inspiration from players." His…

  1. William Brickman, Master Teacher

    ERIC Educational Resources Information Center

    Swing, Elizabeth Sherman

    2010-01-01

    In this article, the author shares her encounter and relationship with William Brickman as her master teacher. William Brickman was her professor, her dissertation advisor, her mentor, and her friend. Her pursuit of a Ph.D. in late middle age may have seemed strange to friends, family, and some of her professors, but not to Brickman. She enrolled…

  2. The gene for replication factor C subunit 2 (RFC2) is within the 7q11.23 Williams syndrome deletion

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Peoples, R.; Perez-Jurado, L.; Francke, U.

    1996-06-01

    Williams syndrome (WS) is a developmental disorder with multiple system manifestations, including supraval var aortic stenosis (SVAS), peripheral pulmonic stenosis, connective tissue abnormalities, short stature, characteristic personality profile and cognitive deficits, and variable hypercalcemia in infancy. It is caused by heterozygosity for a chromosomal deletion of part of band 7q11.23 including the elastin locus (ELN). Since disruption of the ELN gene causes autosomal dominant SVAS, it is assumed that ELN haploinsufficiency is responsible for the cardiovascular features of WS. The deletion that extends from the ELN locus in both directions is {ge}200 kb in size, although estimates of {ge}2 Mbmore » are suggested by high-resolution chromosome banding and physical mapping studies. We have searched for additional dosage-sensitive genes within the deletion that may be responsible for the noncardiovascular features. We report here that the gene for replication factor C subunit 2 (RFC2) maps within the WS deletion region and was found to be deleted in all of 18 WS patients studied. The protein product of RFC2 is part of a multimeric complex involved in DNA elongation during replication. 14 refs., 3 figs.« less

  3. Hemizygosity at the NCF1 Gene in Patients with Williams-Beuren Syndrome Decreases Their Risk of Hypertension

    PubMed Central

    Del Campo, Miguel; Antonell, Anna; Magano, Luis F.; Muñoz, Francisco J.; Flores, Raquel; Bayés, Mònica; Pérez Jurado, Luis A.

    2006-01-01

    Williams-Beuren syndrome (WBS), caused by a heterozygous deletion at 7q11.23, represents a model for studying hypertension, the leading risk factor for mortality worldwide, in a genetically determined disorder. Haploinsufficiency at the elastin gene is known to lead to the vascular stenoses in WBS and is also thought to predispose to hypertension, present in ∼50% of patients. Detailed clinical and molecular characterization of 96 patients with WBS was performed to explore clinical-molecular correlations. Deletion breakpoints were precisely defined and were found to result in variability at two genes, NCF1 and GTF2IRD2. Hypertension was significantly less prevalent in patients with WBS who had the deletion that included NCF1 (P=.02), a gene coding for the p47phox subunit of the NADPH oxidase. Decreased p47phox protein levels, decreased superoxide anion production, and lower protein nitrotyrosination were all observed in cell lines from patients hemizygous at NCF1. Our results indicate that the loss of a functional copy of NCF1 protects a proportion of patients with WBS against hypertension, likely through a lifelong reduced angiotensin II–mediated oxidative stress. Therefore, antioxidant therapy that reduces NADPH oxidase activity might have a potential benefit in identifiable patients with WBS in whom serious complications related to hypertension have been reported, as well as in forms of essential hypertension mediated by a similar pathogenic mechanism. PMID:16532385

  4. Genome-wide identification and expression profiling reveal tissue-specific expression and differentially-regulated genes involved in gibberellin metabolism between Williams banana and its dwarf mutant.

    PubMed

    Chen, Jingjing; Xie, Jianghui; Duan, Yajie; Hu, Huigang; Hu, Yulin; Li, Weiming

    2016-05-27

    Dwarfism is one of the most valuable traits in banana breeding because semi-dwarf cultivars show good resistance to damage by wind and rain. Moreover, these cultivars present advantages of convenient cultivation, management, and so on. We obtained a dwarf mutant '8818-1' through EMS (ethyl methane sulphonate) mutagenesis of Williams banana 8818 (Musa spp. AAA group). Our research have shown that gibberellins (GAs) content in 8818-1 false stems was significantly lower than that in its parent 8818 and the dwarf type of 8818-1 could be restored by application of exogenous GA3. Although GA exerts important impacts on the 8818-1 dwarf type, our understanding of the regulation of GA metabolism during banana dwarf mutant development remains limited. Genome-wide screening revealed 36 candidate GA metabolism genes were systematically identified for the first time; these genes included 3 MaCPS, 2 MaKS, 1 MaKO, 2 MaKAO, 10 MaGA20ox, 4 MaGA3ox, and 14 MaGA2ox genes. Phylogenetic tree and conserved protein domain analyses showed sequence conservation and divergence. GA metabolism genes exhibited tissue-specific expression patterns. Early GA biosynthesis genes were constitutively expressed but presented differential regulation in different tissues in Williams banana. GA oxidase family genes were mainly transcribed in young fruits, thus suggesting that young fruits were the most active tissue involved in GA metabolism, followed by leaves, bracts, and finally approximately mature fruits. Expression patterns between 8818 and 8818-1 revealed that MaGA20ox4, MaGA20ox5, and MaGA20ox7 of the MaGA20ox gene family and MaGA2ox7, MaGA2ox12, and MaGA2ox14 of the MaGA2ox gene family exhibited significant differential expression and high-expression levels in false stems. These genes are likely to be responsible for the regulation of GAs content in 8818-1 false stems. Overall, phylogenetic evolution, tissue specificity and differential expression analyses of GA metabolism genes can provide a

  5. Structural variants in genes associated with human Williams-Beuren syndrome underlie stereotypical hypersociability in domestic dogs.

    PubMed

    vonHoldt, Bridgett M; Shuldiner, Emily; Koch, Ilana Janowitz; Kartzinel, Rebecca Y; Hogan, Andrew; Brubaker, Lauren; Wanser, Shelby; Stahler, Daniel; Wynne, Clive D L; Ostrander, Elaine A; Sinsheimer, Janet S; Udell, Monique A R

    2017-07-01

    Although considerable progress has been made in understanding the genetic basis of morphologic traits (for example, body size and coat color) in dogs and wolves, the genetic basis of their behavioral divergence is poorly understood. An integrative approach using both behavioral and genetic data is required to understand the molecular underpinnings of the various behavioral characteristics associated with domestication. We analyze a 5-Mb genomic region on chromosome 6 previously found to be under positive selection in domestic dog breeds. Deletion of this region in humans is linked to Williams-Beuren syndrome (WBS), a multisystem congenital disorder characterized by hypersocial behavior. We associate quantitative data on behavioral phenotypes symptomatic of WBS in humans with structural changes in the WBS locus in dogs. We find that hypersociability, a central feature of WBS, is also a core element of domestication that distinguishes dogs from wolves. We provide evidence that structural variants in GTF2I and GTF2IRD1 , genes previously implicated in the behavioral phenotype of patients with WBS and contained within the WBS locus, contribute to extreme sociability in dogs. This finding suggests that there are commonalities in the genetic architecture of WBS and canine tameness and that directional selection may have targeted a unique set of linked behavioral genes of large phenotypic effect, allowing for rapid behavioral divergence of dogs and wolves, facilitating coexistence with humans.

  6. Smaller and larger deletions of the Williams Beuren syndrome region implicate genes involved in mild facial phenotype, epilepsy and autistic traits.

    PubMed

    Fusco, Carmela; Micale, Lucia; Augello, Bartolomeo; Teresa Pellico, Maria; Menghini, Deny; Alfieri, Paolo; Cristina Digilio, Maria; Mandriani, Barbara; Carella, Massimo; Palumbo, Orazio; Vicari, Stefano; Merla, Giuseppe

    2014-01-01

    Williams Beuren syndrome (WBS) is a multisystemic disorder caused by a hemizygous deletion of 1.5 Mb on chromosome 7q11.23 spanning 28 genes. A few patients with larger and smaller WBS deletion have been reported. They show clinical features that vary between isolated SVAS to the full spectrum of WBS phenotype, associated with epilepsy or autism spectrum behavior. Here we describe four patients with atypical WBS 7q11.23 deletions. Two carry ~3.5 Mb larger deletion towards the telomere that includes Huntingtin-interacting protein 1 (HIP1) and tyrosine 3-monooxygenase/tryptophan 5-monooxigenase activation protein gamma (YWHAG) genes. Other two carry a shorter deletion of ~1.2 Mb at centromeric side that excludes the distal WBS genes BAZ1B and FZD9. Along with previously reported cases, genotype-phenotype correlation in the patients described here further suggests that haploinsufficiency of HIP1 and YWHAG might cause the severe neurological and neuropsychological deficits including epilepsy and autistic traits, and that the preservation of BAZ1B and FZD9 genes may be related to mild facial features and moderate neuropsychological deficits. This report highlights the importance to characterize additional patients with 7q11.23 atypical deletions comparing neuropsychological and clinical features between these individuals to shed light on the pathogenic role of genes within and flanking the WBS region.

  7. A Diagnosis to Consider in an Adult Patient with Facial Features and Intellectual Disability: Williams Syndrome.

    PubMed

    Doğan, Özlem Akgün; Şimşek Kiper, Pelin Özlem; Utine, Gülen Eda; Alikaşifoğlu, Mehmet; Boduroğlu, Koray

    2017-03-01

    Williams syndrome (OMIM #194050) is a rare, well-recognized, multisystemic genetic condition affecting approximately 1/7,500 individuals. There are no marked regional differences in the incidence of Williams syndrome. The syndrome is caused by a hemizygous deletion of approximately 28 genes, including ELN on chromosome 7q11.2. Prenatal-onset growth retardation, distinct facial appearance, cardiovascular abnormalities, and unique hypersocial behavior are among the most common clinical features. Here, we report the case of a patient referred to us with distinct facial features and intellectual disability, who was diagnosed with Williams syndrome at the age of 37 years. Our aim is to increase awareness regarding the diagnostic features and complications of this recognizable syndrome among adult health care providers. Williams syndrome is usually diagnosed during infancy or childhood, but in the absence of classical findings, such as cardiovascular anomalies, hypercalcemia, and cognitive impairment, the diagnosis could be delayed. Due to the multisystemic and progressive nature of the syndrome, accurate diagnosis is critical for appropriate care and screening for the associated morbidities that may affect the patient's health and well-being.

  8. Handedness and corpus callosal morphology in Williams syndrome.

    PubMed

    Martens, Marilee A; Wilson, Sarah J; Chen, Jian; Wood, Amanda G; Reutens, David C

    2013-02-01

    Williams syndrome is a neurodevelopmental genetic disorder caused by a hemizygous deletion on chromosome 7q11.23, resulting in atypical brain structure and function, including abnormal morphology of the corpus callosum. An influence of handedness on the size of the corpus callosum has been observed in studies of typical individuals, but handedness has not been taken into account in studies of callosal morphology in Williams syndrome. We hypothesized that callosal area is smaller and the size of the splenium and isthmus is reduced in individuals with Williams syndrome compared to healthy controls, and examined age, sex, and handedness effects on corpus callosal area. Structural magnetic resonance imaging scans were obtained on 25 individuals with Williams syndrome (18 right-handed, 7 left-handed) and 25 matched controls. We found that callosal thickness was significantly reduced in the splenium of Williams syndrome individuals compared to controls. We also found novel evidence that the callosal area was smaller in left-handed participants with Williams syndrome than their right-handed counterparts, with opposite findings observed in the control group. This novel finding may be associated with LIM-kinase hemizygosity, a characteristic of Williams syndrome. The findings may have significant clinical implications in future explorations of the Williams syndrome cognitive phenotype.

  9. 33 CFR 167.1700 - In Prince William Sound: General.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 33 Navigation and Navigable Waters 2 2014-07-01 2014-07-01 false In Prince William Sound: General... Schemes and Precautionary Areas Pacific West Coast § 167.1700 In Prince William Sound: General. The Prince William Sound Traffic Separation Scheme consists of four parts: Prince William Sound Traffic Separation...

  10. 33 CFR 167.1700 - In Prince William Sound: General.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 33 Navigation and Navigable Waters 2 2013-07-01 2013-07-01 false In Prince William Sound: General... Schemes and Precautionary Areas Pacific West Coast § 167.1700 In Prince William Sound: General. The Prince William Sound Traffic Separation Scheme consists of four parts: Prince William Sound Traffic Separation...

  11. 33 CFR 167.1700 - In Prince William Sound: General.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 33 Navigation and Navigable Waters 2 2012-07-01 2012-07-01 false In Prince William Sound: General... Schemes and Precautionary Areas Pacific West Coast § 167.1700 In Prince William Sound: General. The Prince William Sound Traffic Separation Scheme consists of four parts: Prince William Sound Traffic Separation...

  12. 33 CFR 167.1700 - In Prince William Sound: General.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 33 Navigation and Navigable Waters 2 2011-07-01 2011-07-01 false In Prince William Sound: General... Schemes and Precautionary Areas Pacific West Coast § 167.1700 In Prince William Sound: General. The Prince William Sound Traffic Separation Scheme consists of four parts: Prince William Sound Traffic Separation...

  13. Robin Williams' suicide: a case study.

    PubMed

    Tohid, Hassaan

    2016-01-01

    The world renowned comedian and four-time Oscar nominated actor Robin Williams died on August 11, 2014. From the outset, the news indicated that his death was believed to be a suicide and this was later confirmed to be true by the autopsy reports. Williams had been suffering from severe depression, which is believed to be the leading contributor to his suicide. In this case study, I will highlight the event of the actor's suicide and the main risk factors along with depression leading to his tragic death. As of the end of 2015, no other case study seemed to have addressed or explored the links between the cause (or causes) and events leading to Robin Williams' suicide. Robin Williams was suffering from relationship problems, financial problems, drug addiction, and major depression. All of these factors led to his suicide. The chances of committing suicide drastically increase in the presence of any of the key risk factors. Unfortunately, the actor Robin Williams was dealing with four of the major risk factors all together, which put him at a high risk of committing suicide and eventually led to his tragic death.

  14. Activity of genes with functions in human Williams-Beuren Syndrome are impacted by mobile element insertions in the gray wolf genome.

    PubMed

    vonHoldt, Bridgett M; Ji, Sarah S; Aardema, Matthew L; Stahler, Daniel; Udell, Monique A R; Sinsheimer, Janet S

    2018-06-01

    In canines, transposon dynamics have been associated with a hyper-social behavioral syndrome, although the functional mechanism has yet to be described. We investigate the epigenetic and transcriptional consequences of these behavior-associated mobile element insertions in dogs and Yellowstone wolves. We posit that the transposons themselves may not be the causative feature; rather, their transcriptional regulation may exert the functional impact. We survey four outlier transposons associated with hyper-sociability, with the expectation that they are targeted for epigenetic silencing. We predict hyper-methylation of mobile element insertions (MEIs), suggestive that the epigenetic silencing of and not the MEIs themselves may be driving dysregulation of nearby genes. We found that transposon-derived sequences are significantly hyper-methylated, regardless of their copy number or species. Further, we have assessed transcriptome sequence data and found evidence that mobile element insertions impact the expression levels of six genes (WBSCR17, LIMK1, GTF2I, WBSCR27, BAZ1B, and BCL7B), all of which have known roles in human Williams-Beuren syndrome due to changes in copy number, typically hemizygosity. Although further evidence is needed, our results suggest that a few insertions alter local expression at multiple genes, likely through a cis-regulatory mechanism that excludes proximal methylation.

  15. Conceptualizing neurodevelopmental disorders through a mechanistic understanding of fragile X syndrome and Williams syndrome

    PubMed Central

    Fung, Lawrence K.; Quintin, Eve-Marie; Haas, Brian W.

    2013-01-01

    Purpose of review The overarching goal of this review is to compare and contrast the cognitive-behavioral features of fragile X syndrome (FraX) and Williams syndrome and to review the putative neural and molecular underpinnings of these features. Information is presented in a framework that provides guiding principles for conceptualizing gene-brain-behavior associations in neurodevelopmental disorders. Recent findings Abnormalities, in particular cognitive-behavioral domains with similarities in underlying neurodevelopmental correlates, occur in both FraX and Williams syndrome including aberrant frontostriatal pathways leading to executive function deficits, and magnocellular/dorsal visual stream, superior parietal lobe, inferior parietal lobe, and postcentral gyrus abnormalities contributing to deficits in visuospatial function. Compelling cognitive–behavioral and neurodevelopmental contrasts also exist in these two disorders, for example, aberrant amygdala and fusiform cortex structure and function occurring in the context of contrasting social behavioral phenotypes, and temporal cortical and cerebellar abnormalities potentially underlying differences in language function. Abnormal dendritic development is a shared neurodevelopmental morphologic feature between FraX and Williams syndrome. Commonalities in molecular machinery and processes across FraX and Williams syndrome occur as well – microRNAs involved in translational regulation of major synaptic proteins; scaffolding proteins in excitatory synapses; and proteins involved in axonal development. Summary Although the genetic variations leading to FraX and Williams syndrome are different, important similarities and contrasts in the phenotype, neurocircuitry, molecular machinery, and cellular processes in these two disorders allow for a unique approach to conceptualizing gene–brain–behavior links occurring in neurodevelopmental disorders. PMID:22395002

  16. Neural correlates of genetically abnormal social cognition in Williams syndrome.

    PubMed

    Meyer-Lindenberg, Andreas; Hariri, Ahmad R; Munoz, Karen E; Mervis, Carolyn B; Mattay, Venkata S; Morris, Colleen A; Berman, Karen Faith

    2005-08-01

    Williams-Beuren syndrome (WBS), caused by a microdeletion of approximately 21 genes on chromosome 7q11.23, is characterized by unique hypersociability combined with increased non-social anxiety. Using functional neuroimaging, we found reduced amygdala activation in individuals with WBS for threatening faces but increased activation for threatening scenes, relative to matched normal controls. Activation and interactions of prefrontal regions linked to amygdala, especially orbitofrontal cortex, were abnormal, suggesting a genetically controlled neural circuitry for regulating human social behavior.

  17. 33 CFR 110.233 - Prince William Sound, Alaska.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 33 Navigation and Navigable Waters 1 2014-07-01 2014-07-01 false Prince William Sound, Alaska. 110... ANCHORAGES ANCHORAGE REGULATIONS Anchorage Grounds § 110.233 Prince William Sound, Alaska. (a) The anchorage grounds. In Prince William Sound, Alaska, beginning at a point at latitude 60°40′00″ N., longitude 146°40...

  18. 33 CFR 110.233 - Prince William Sound, Alaska.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 33 Navigation and Navigable Waters 1 2012-07-01 2012-07-01 false Prince William Sound, Alaska. 110... ANCHORAGES ANCHORAGE REGULATIONS Anchorage Grounds § 110.233 Prince William Sound, Alaska. (a) The anchorage grounds. In Prince William Sound, Alaska, beginning at a point at latitude 60°40′00″ N., longitude 146°40...

  19. 33 CFR 110.233 - Prince William Sound, Alaska.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 33 Navigation and Navigable Waters 1 2013-07-01 2013-07-01 false Prince William Sound, Alaska. 110... ANCHORAGES ANCHORAGE REGULATIONS Anchorage Grounds § 110.233 Prince William Sound, Alaska. (a) The anchorage grounds. In Prince William Sound, Alaska, beginning at a point at latitude 60°40′00″ N., longitude 146°40...

  20. 33 CFR 110.233 - Prince William Sound, Alaska.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 33 Navigation and Navigable Waters 1 2010-07-01 2010-07-01 false Prince William Sound, Alaska. 110... ANCHORAGES ANCHORAGE REGULATIONS Anchorage Grounds § 110.233 Prince William Sound, Alaska. (a) The anchorage grounds. In Prince William Sound, Alaska, beginning at a point at latitude 60°40′00″ N., longitude 146°40...

  1. 33 CFR 110.233 - Prince William Sound, Alaska.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 33 Navigation and Navigable Waters 1 2011-07-01 2011-07-01 false Prince William Sound, Alaska. 110... ANCHORAGES ANCHORAGE REGULATIONS Anchorage Grounds § 110.233 Prince William Sound, Alaska. (a) The anchorage grounds. In Prince William Sound, Alaska, beginning at a point at latitude 60°40′00″ N., longitude 146°40...

  2. 77 FR 45331 - Prince William Sound Resource Advisory Committee

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-07-31

    ... DEPARTMENT OF AGRICULTURE Forest Service Prince William Sound Resource Advisory Committee AGENCY: Forest Service, USDA. ACTION: Notice of meeting. SUMMARY: The Prince William Sound Resource Advisory... Prince William Sound Resource Advisory Committee (RAC) will be discussing and voting on proposals that...

  3. 76 FR 1130 - Prince William Sound Resource Advisory Committee

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-01-07

    ... DEPARTMENT OF AGRICULTURE Forest Service Prince William Sound Resource Advisory Committee AGENCY: Forest Service, USDA. ACTION: Notice of meeting. SUMMARY: The Prince William Sound Resource Advisory... conducted: The Prince William Sound Resource Advisory Committee (RAC) will be discussing and voting on...

  4. Maniac Talk - Dr. William Lau

    NASA Image and Video Library

    2014-01-24

    William Lau Maniac Lecture, 24 January, 2014 Dr. William Lau, Deputy Director for Atmospheres, Earth Science Division at NASA Goddard, presented a Maniac Talk entitled "My Story: A Tale of Three Continents." Bill shared his early childhood under a colonial education system with strong Chinese cultural influence and how world events, cultural and education system of three major continents, Europe, Asia and North America shaped his upbringing career goals and work ethics.

  5. 76 FR 18715 - Prince William Sound Resource Advisory Committee

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-04-05

    ... DEPARTMENT OF AGRICULTURE Forest Service Prince William Sound Resource Advisory Committee AGENCY: Forest Service, USDA. ACTION: Notice of meeting. SUMMARY: The Prince William Sound Resource Advisory... meeting is open to the public. The following business will be conducted: The Prince William Sound Resource...

  6. 33 CFR 167.1700 - In Prince William Sound: General.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... (CONTINUED) PORTS AND WATERWAYS SAFETY OFFSHORE TRAFFIC SEPARATION SCHEMES Description of Traffic Separation Schemes and Precautionary Areas Pacific West Coast § 167.1700 In Prince William Sound: General. The Prince William Sound Traffic Separation Scheme consists of four parts: Prince William Sound Traffic Separation...

  7. William Russell on Schools in Bulgaria

    ERIC Educational Resources Information Center

    Popov, Nikolay; Sabic-El-Rayess, Amra

    2013-01-01

    William Russell became one of the most influential educators in the field of international and comparative education in the first half of the 20th century. In 1914, William Russell obtained his PhD from Teachers College and, within few years, became a prominent figure internationally. He traveled through Europe and taught in Japan and Siberia, as…

  8. The social phenotype of Williams syndrome.

    PubMed

    Järvinen, Anna; Korenberg, Julie R; Bellugi, Ursula

    2013-06-01

    Williams syndrome (WS) offers an exciting model for social neuroscience because its genetic basis is well-defined, and the unique phenotype reflects dimensions of prosocial behaviors. WS is associated with a strong drive to approach strangers, a gregarious personality, heightened social engagement yet difficult peer interactions, high nonsocial anxiety, unusual bias toward positive affect, and diminished sensitivity to fear. New neurobiological evidence points toward alterations in structure, function, and connectivity of the social brain (amygdala, fusiform face area, orbital-frontal regions). Recent genetic studies implicate gene networks in the WS region with the dysregulation of prosocial neuropeptides. The study of WS has implications for understanding human social development, and may provide insight for translating genetic and neuroendocrine evidence into treatments for disorders of social behavior. Copyright © 2013 Elsevier Ltd. All rights reserved.

  9. Williams-Beuren's Syndrome: A Case Report.

    PubMed

    Zamani, Hassan; Babazadeh, Kazem; Fattahi, Saeid; Mokhtari-Esbuie, Farzad

    2012-01-01

    Williams-Beuren syndrome is a rare familial multisystem disorder occurring in 1 per 20,000 live births. It is characterized by congenital heart defects (CHD), skeletal and renal anomalies, cognitive disorder, social personality disorder and dysmorphic facies. We present a case of Williams syndrome that presented to us with heart murmur and cognitive problem. A 5-year-old girl referred to pediatric cardiologist because of heart murmurs. She had a systolic murmur (2-3/6) in right upper sternal border with radiation to right cervical region. She also had a bulge forehead. Angiography showed mild supra valvular aortic stenosis and mild multiple peripheral pulmonary stenosis. Fluorescent in situ hybridization (FISH) was performed and the result was: 46.XX, ish del (7q11.2) (ELN X1) (7q22 X2) ELN deletion compatible with Williams syndrome. Peripheral pulmonary artery stenosis is associated with Noonan syndrome, Alagille syndrome, Cutis laxa, Ehler-Danlos syndrome, and Silver-Russel syndrome. The patient had peripheral pulmonary artery stenosis, but no other signs of these syndromes were present, and also she had a supravalvular aortic stenosis which was not seen in other syndromes except Williams syndrome. Conclusion. According to primary symptoms, paraclinical and clinical finding such as dysmorphic facies, cognitive disorder and congenital heart defect, Williams syndrome was the first diagnosis. We suggest a more attention for evaluating heart murmur in childhood period, especially when the patient has abnormal facial features or mental problem.

  10. Williams-Beuren's Syndrome: A Case Report

    PubMed Central

    Zamani, Hassan; Babazadeh, Kazem; Fattahi, Saeid; Mokhtari-Esbuie, Farzad

    2012-01-01

    Williams-Beuren syndrome is a rare familial multisystem disorder occurring in 1 per 20,000 live births. It is characterized by congenital heart defects (CHD), skeletal and renal anomalies, cognitive disorder, social personality disorder and dysmorphic facies. We present a case of Williams syndrome that presented to us with heart murmur and cognitive problem. A 5-year-old girl referred to pediatric cardiologist because of heart murmurs. She had a systolic murmur (2-3/6) in right upper sternal border with radiation to right cervical region. She also had a bulge forehead. Angiography showed mild supra valvular aortic stenosis and mild multiple peripheral pulmonary stenosis. Fluorescent in situ hybridization (FISH) was performed and the result was: 46.XX, ish del (7q11.2) (ELN X1) (7q22 X2) ELN deletion compatible with Williams syndrome. Peripheral pulmonary artery stenosis is associated with Noonan syndrome, Alagille syndrome, Cutis laxa, Ehler-Danlos syndrome, and Silver-Russel syndrome. The patient had peripheral pulmonary artery stenosis, but no other signs of these syndromes were present, and also she had a supravalvular aortic stenosis which was not seen in other syndromes except Williams syndrome. Conclusion. According to primary symptoms, paraclinical and clinical finding such as dysmorphic facies, cognitive disorder and congenital heart defect, Williams syndrome was the first diagnosis. We suggest a more attention for evaluating heart murmur in childhood period, especially when the patient has abnormal facial features or mental problem. PMID:22927862

  11. Structural controls on ground-water conditions and estimated aquifer properties near Bill Williams Mountain, Williams, Arizona

    USGS Publications Warehouse

    Pierce, Herbert A.

    2001-01-01

    As of 1999, surface water collected and stored in reservoirs is the sole source of municipal water for the city of Williams. During 1996 and 1999, reservoirs reached historically low levels. Understanding the ground-water flow system is critical to managing the ground-water resources in this part of the Coconino Plateau. The nearly 1,000-meter-deep regional aquifer in the Redwall and Muav Limestones, however, makes studying or utilizing the resource difficult. Near-vertical faults and complex geologic structures control the ground-water flow system on the southwest side of the Kaibab Uplift near Williams, Arizona. To address the hydrogeologic complexities in the study area, a suite of techniques, which included aeromagnetic, gravity, square-array resistivity, and audiomagnetotelluric surveys, were applied as part of a regional study near Bill Williams Mountain. Existing well data and interpreted geophysical data were compiled and used to estimate depths to the water table and to prepare a potentiometric map. Geologic characteristics, such as secondary porosity, coefficient of anisotropy, and fracture-strike direction, were calculated at several sites to examine how these characteristics change with depth. The 14-kilometer-wide, seismically active northwestward-trending Cataract Creek and the northeastward-trending Mesa Butte Fault systems intersect near Bill Williams Mountain. Several north-south-trending faults may provide additional block faulting north and west of Bill Williams Mountain. Because of the extensive block faulting and regional folding, the volcanic and sedimentary rocks are tilted toward one or more of these faults. These faults provide near-vertical flow paths to the regional water table. The nearly radial fractures allow water that reaches the regional aquifer to move away from the Bill Williams Mountain area. Depth to the regional aquifer is highly variable and depends on location and local structures. On the basis of interpreted

  12. [Diagnosis of a case with Williams-Beuren syndrome with nephrocalcinosis using chromosome microarray analysis].

    PubMed

    Jin, S J; Liu, M; Long, W J; Luo, X P

    2016-12-02

    Objective: To explore the clinical phenotypes and the genetic cause for a boy with unexplained growth retardation, nephrocalcinosis, auditory anomalies and multi-organ/system developmental disorders. Method: Routine G-banding and chromosome microarray analysis were applied to a child with unexplained growth retardation, nephrocalcinosis, auditory anomalies and multi-organ/system developmental disorders treated in the Department of Pediatrics of Tongji Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology in September 2015 and his parents to conduct the chromosomal karyotype analysis and the whole genome scanning. Deleted genes were searched in the Decipher and NCBI databases, and their relationships with the clinical phenotypes were analyzed. Result: A six-month-old boy was refered to us because of unexplained growth retardation and feeding intolerance.The affected child presented with abnormal manifestation such as special face, umbilical hernia, growth retardation, hypothyroidism, congenital heart disease, right ear sensorineural deafness, hypercalcemia and nephrocalcinosis. The child's karyotype was 46, XY, 16qh + , and his parents' karyotypes were normal. Chromosome microarray analysis revealed a 1 436 kb deletion on the 7q11.23(72701098_74136633) region of the child. This region included 23 protein-coding genes, which were reported to be corresponding to Williams-Beuren syndrome and its certain clinical phenotypes. His parents' results of chromosome microarray analysis were normal. Conclusion: A boy with characteristic manifestation of Williams-Beuren syndrome and rare nephrocalcinosis was diagnosed using chromosome microarray analysis. The deletion on the 7q11.23 might be related to the clinical phenotypes of Williams-Beuren syndrome, yet further studies are needed.

  13. Bilateral vocal cord paralysis and hypothyroidism as presenting symptoms of Williams-Beuren syndrome: a case report.

    PubMed

    Koren, Ilana; Kessel, Ira; Rotschild, Avi; Cohen-Kerem, Raanan

    2015-09-01

    Williams-Beuren syndrome is a rare neurodevelopmental disorder caused by deletion of 1.5-1.8Mb genes on chromosome 7q11.23. The syndrome was first described as a triad of supra-valvular aortic stenosis, mental retardation, and distinctive facial features. Our patient was referred due to audible inspiratory stridor when he was seven days old. Following endoscopy he was diagnosed with bilateral vocal cord paralysis and was eventually intubated due to respiratory de-compensation followed by tracheotomy. On further workup he was diagnosed with hypothyroidism. Genetic workup supported the diagnosis of Williams-Beuren syndrome. We report here a case with an unusual clinical presentation. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  14. A human neurodevelopmental model for Williams syndrome

    PubMed Central

    Chailangkarn, Thanathom; Trujillo, Cleber A.; Freitas, Beatriz C.; Hrvoj-Mihic, Branka; Herai, Roberto H.; Yu, Diana X.; Brown, Timothy T.; Marchetto, Maria C. N.; Bardy, Cedric; McHenry, Lauren; Stefanacci, Lisa; Järvinen, Anna; Searcy, Yvonne M.; DeWitt, Michelle; Wong, Wenny; Lai, Philip; Ard, M. Colin; Hanson, Kari L.; Romero, Sarah; Jacobs, Bob; Dale, Anders M.; Dai, Li; Korenberg, Julie R.; Gage, Fred H.; Bellugi, Ursula; Halgren, Eric; Semendeferi, Katerina; Muotri, Alysson R.

    2016-01-01

    Summary Williams syndrome (WS) is a genetic neurodevelopmental disorder characterized by an uncommon hypersociability and a mosaic of retained and compromised linguistic and cognitive abilities. Nearly all clinically diagnosed individuals with WS lack precisely the same set of genes, with breakpoints in chromosome band 7q11.231–5. The contribution of specific genes to the neuroanatomical and functional alterations, leading to behavioral pathologies in humans, remains largely unexplored. Here, we investigate neural progenitor cells (NPCs) and cortical neurons derived from WS and typically developing (TD) induced pluripotent stem cells (iPSCs). WS NPCs have an increased doubling time and apoptosis compared to TD NPCs. Using an atypical WS subject6, 7, we narrowed this cellular phenotype to a single gene candidate, FZD9. At the neuronal stage, WS-derived layers V/VI cortical neurons were characterized by longer total dendrites, increased numbers of spines and synapses, aberrant calcium oscillation and altered network connectivity. Morphometric alterations observed in WS neurons were validated after Golgi staining of postmortem layers V/VI cortical neurons. This human iPSC model8 fills in the current knowledge gap in WS cellular biology and could lead to further insights into the molecular mechanism underlying the disorder and the human social brain. PMID:27509850

  15. Inversion of the Williams syndrome region is a common polymorphism found more frequently in parents of children with Williams syndrome.

    PubMed

    Hobart, Holly H; Morris, Colleen A; Mervis, Carolyn B; Pani, Ariel M; Kistler, Doris J; Rios, Cecilia M; Kimberley, Kendra W; Gregg, Ronald G; Bray-Ward, Patricia

    2010-05-15

    Williams syndrome (WS) is a multisystem disorder caused by deletion of about 1.55 Mb of DNA (including 26 genes) on chromosome 7q11.23, a region predisposed to recombination due to its genomic structure. Deletion of the Williams syndrome chromosome region (WSCR) occurs sporadically. To better define chance for familial recurrence and to investigate the prevalence of genomic rearrangements of the region, 257 children with WS and their parents were studied. We determined deletion size in probands by metaphase FISH, parent-of-origin of the deleted chromosome by molecular genetic methods, and inversion status of the WSCR in both parents by interphase FISH. The frequency of WSCR inversion in the transmitting parent group was 24.9%. In contrast, the rate of inversion in the non-transmitting parent group (a reasonable estimate of the rate in the general population) was 5.8%. There were no significant gender differences with respect to parent-of-origin for the deleted chromosome or the incidence of the inversion polymorphism. There was no difference in the rate of spontaneous abortion for mothers heterozygous for the WSCR inversion relative to mothers without the inversion. We calculate that for a parent heterozygous for a WSCR inversion, the chance to have a child with WS is about 1 in 1,750, in contrast to the 1 in 9,500 chance for a parent without an inversion.

  16. 75 FR 39910 - Prince William Sound Resource Advisory Committee; Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-07-13

    ... DEPARTMENT OF AGRICULTURE Forest Service Prince William Sound Resource Advisory Committee; Meeting AGENCY: Forest Service, USDA. ACTION: Notice of meeting. SUMMARY: The Prince William Sound Resource..., Anchorage, Alaska 99503. Send written comments to Prince William Sound Resource Advisory Committee, c/o USDA...

  17. Social Cognition in Williams Syndrome: Face Tuning

    PubMed Central

    Pavlova, Marina A.; Heiz, Julie; Sokolov, Alexander N.; Barisnikov, Koviljka

    2016-01-01

    Many neurological, neurodevelopmental, neuropsychiatric, and psychosomatic disorders are characterized by impairments in visual social cognition, body language reading, and facial assessment of a social counterpart. Yet a wealth of research indicates that individuals with Williams syndrome exhibit remarkable concern for social stimuli and face fascination. Here individuals with Williams syndrome were presented with a set of Face-n-Food images composed of food ingredients and in different degree resembling a face (slightly bordering on the Giuseppe Arcimboldo style). The primary advantage of these images is that single components do not explicitly trigger face-specific processing, whereas in face images commonly used for investigating face perception (such as photographs or depictions), the mere occurrence of typical cues already implicates face presence. In a spontaneous recognition task, participants were shown a set of images in a predetermined order from the least to most resembling a face. Strikingly, individuals with Williams syndrome exhibited profound deficits in recognition of the Face-n-Food images as a face: they did not report seeing a face on the images, which typically developing controls effortlessly recognized as a face, and gave overall fewer face responses. This suggests atypical face tuning in Williams syndrome. The outcome is discussed in the light of a general pattern of social cognition in Williams syndrome and brain mechanisms underpinning face processing. PMID:27531986

  18. Social Cognition in Williams Syndrome: Face Tuning.

    PubMed

    Pavlova, Marina A; Heiz, Julie; Sokolov, Alexander N; Barisnikov, Koviljka

    2016-01-01

    Many neurological, neurodevelopmental, neuropsychiatric, and psychosomatic disorders are characterized by impairments in visual social cognition, body language reading, and facial assessment of a social counterpart. Yet a wealth of research indicates that individuals with Williams syndrome exhibit remarkable concern for social stimuli and face fascination. Here individuals with Williams syndrome were presented with a set of Face-n-Food images composed of food ingredients and in different degree resembling a face (slightly bordering on the Giuseppe Arcimboldo style). The primary advantage of these images is that single components do not explicitly trigger face-specific processing, whereas in face images commonly used for investigating face perception (such as photographs or depictions), the mere occurrence of typical cues already implicates face presence. In a spontaneous recognition task, participants were shown a set of images in a predetermined order from the least to most resembling a face. Strikingly, individuals with Williams syndrome exhibited profound deficits in recognition of the Face-n-Food images as a face: they did not report seeing a face on the images, which typically developing controls effortlessly recognized as a face, and gave overall fewer face responses. This suggests atypical face tuning in Williams syndrome. The outcome is discussed in the light of a general pattern of social cognition in Williams syndrome and brain mechanisms underpinning face processing.

  19. 33 CFR 161.60 - Vessel Traffic Service Prince William Sound.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... William Sound. 161.60 Section 161.60 Navigation and Navigable Waters COAST GUARD, DEPARTMENT OF HOMELAND... Movement Reporting System Areas and Reporting Points § 161.60 Vessel Traffic Service Prince William Sound... Cape Hinchinbrook Light to Schooner Rock Light, comprising that portion of Prince William Sound between...

  20. 33 CFR 161.60 - Vessel Traffic Service Prince William Sound.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... William Sound. 161.60 Section 161.60 Navigation and Navigable Waters COAST GUARD, DEPARTMENT OF HOMELAND... Movement Reporting System Areas and Reporting Points § 161.60 Vessel Traffic Service Prince William Sound... Cape Hinchinbrook Light to Schooner Rock Light, comprising that portion of Prince William Sound between...

  1. 33 CFR 161.60 - Vessel Traffic Service Prince William Sound.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... William Sound. 161.60 Section 161.60 Navigation and Navigable Waters COAST GUARD, DEPARTMENT OF HOMELAND... Movement Reporting System Areas and Reporting Points § 161.60 Vessel Traffic Service Prince William Sound... Cape Hinchinbrook Light to Schooner Rock Light, comprising that portion of Prince William Sound between...

  2. 33 CFR 161.60 - Vessel Traffic Service Prince William Sound.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... William Sound. 161.60 Section 161.60 Navigation and Navigable Waters COAST GUARD, DEPARTMENT OF HOMELAND... Movement Reporting System Areas and Reporting Points § 161.60 Vessel Traffic Service Prince William Sound... Cape Hinchinbrook Light to Schooner Rock Light, comprising that portion of Prince William Sound between...

  3. 33 CFR 161.60 - Vessel Traffic Service Prince William Sound.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... William Sound. 161.60 Section 161.60 Navigation and Navigable Waters COAST GUARD, DEPARTMENT OF HOMELAND... Movement Reporting System Areas and Reporting Points § 161.60 Vessel Traffic Service Prince William Sound... Cape Hinchinbrook Light to Schooner Rock Light, comprising that portion of Prince William Sound between...

  4. Skin findings in Williams syndrome.

    PubMed

    Kozel, Beth A; Bayliss, Susan J; Berk, David R; Waxler, Jessica L; Knutsen, Russell H; Danback, Joshua R; Pober, Barbara R

    2014-09-01

    Previous examination in a small number of individuals with Williams syndrome (also referred to as Williams-Beuren syndrome) has shown subtly softer skin and reduced deposition of elastin, an elastic matrix protein important in tissue recoil. No quantitative information about skin elasticity in individuals with Williams syndrome is available; nor has there been a complete report of dermatologic findings in this population. To fill this knowledge gap, 94 patients with Williams syndrome aged 7-50 years were recruited as part of the skin and vascular elasticity (WS-SAVE) study. They underwent either a clinical dermatologic assessment by trained dermatologists (2010 WSA family meeting) or measurement of biomechanical properties of the skin with the DermaLab™ suction cup (2012 WSA family meeting). Clinical assessment confirmed that soft skin is common in this population (83%), as is premature graying of the hair (80% of those 20 years or older), while wrinkles (92%), and abnormal scarring (33%) were detected in larger than expected proportions. Biomechanical studies detected statistically significant differences in dP (the pressure required to lift the skin), dT (the time required to raise the skin through a prescribed gradient), VE (viscoelasticity), and E (Young's modulus) relative to matched controls. The RT (retraction time) also trended longer but was not significant. The biomechanical differences noted in these patients did not correlate with the presence of vascular defects also attributable to elastin insufficiency (vascular stiffness, hypertension, and arterial stenosis) suggesting the presence of tissue specific modifiers that modulate the impact of elastin insufficiency in each tissue. © 2014 Wiley Periodicals, Inc.

  5. Anterior segment dysgenesis associated with Williams-Beuren syndrome: a case report and review of the literature.

    PubMed

    Todorova, Margarita G; Grieshaber, Matthias C; Cámara, Rafael J A; Miny, Peter; Palmowski-Wolfe, Anja M

    2014-05-21

    Williams-Beuren syndrome is characterized by mild mental retardation, specific neurocognitive profile, hypercalcemia during infancy, distinctive facial features and cardiovascular diseases. We report on complete ophthalmologic, sonographic and genetic evaluation of a girl with a clinical phenotype of Williams-Beuren syndrome, associated with unilateral anterior segment dysgenesis and bilateral cleft of the soft and hard palate. These phenotypic features have not been linked to the haploinsufficiency of genes involved in the microdeletion. A term born girl presented at the initial examination with clouding of the right cornea. On ultrasound biomicroscopy the anterior chamber structures were difficult to differentiate, showing severe adhesions from the opacified cornea to the iris with a kerato-irido-lenticular contact to the remnant lens, a finding consistent with Peters' anomaly. Genetic analyses including FISH confirmed a loss of the critical region 7q11.23, usually associated with the typical Williams-Beuren syndrome. Microsatellite analysis showed a loss of about 2.36 Mb. A diagnosis of Williams-Beuren syndrome was made based on the microdeletion of 7q11.23. The unique features, including unilateral microphthalmia and anterior segment dysgenesis, were unlikely to be caused by the microdeletion. Arguments in favor of the latter are unilateral manifestation, as well as the fact that numerous patients with deletions of comparable or microscopically visible size have not shown similar manifestations.

  6. Williams during EVA 36

    NASA Image and Video Library

    2016-08-19

    Extravehicular crewmember 1 (EV1) Jeff Williams pauses for a photo after installing a Hemispherical (Hemi) Reflector Cover on Pressurized Mating Adapter 2 (PMA-2) during Extravehicular Activity 36 (EVA 36).

  7. Walter C. Williams

    NASA Technical Reports Server (NTRS)

    1949-01-01

    Walter C. Williams arrived from the National Advisory Committee for Aeronautics, Langley Memorial Aeronautical Laboratory, Hampton, Virginia, on September 30, 1946, at the Muroc Army Air Field. He had been named the engineer-in-charge of the small group of five that came with him to the Rogers Dry Lakebed to take part in research flights of a joint NACA-Army Air Forces program involving the rocket-powered Bell XS-1. This established the first permanent National Advisory Committee for Aeronautics presence at the Mojave Desert site in California. This small group grew in numbers to 27 and received permanent status as the NACA Muroc Flight Test Unit from Hugh L. Dryden, NACA's Director of Research, on September 27, 1947. Walt was named Head of the Unit. On November 14, 1949, the Unit along with the 100 employees became the NACA High-Speed Flight Research Station with Walt Williams as Chief. Next came the move from the South Base site to the new headquarters, Bldg. 4800 on the north-west shore of the Rogers Dry lakebed on the Edwards Air Force Base complex. July 1, 1954 saw another name change to the NACA High-Speed Flight Station with Walt remaining the Chief to a complement of about 225 employees. Williams had received a Bachelor of Science Degree in aeronautical engineering from Louisiana State University, Baton Rouge, Louisiana, in 1939. After graduation, he was employed by the Glenn L. Martin Company of Baltimore, Maryland, and later that same year joined the staff of the NACA Langley Memorial Aeronautical Laboratory, where he worked as an engineer in the Flight Division. During the period from September 1946 to July 1954 Williams supervised the activities of several research projects. These included the first successful rocket-powered flight of the XS-1 made by Bell pilot Chalmers Goodlin on December 9, 1946; the record breaking flight of A.F. Captain Chuck Yeager on October 14, 1947, that exceeded the speed of sound; and the first flight of the jet

  8. Metabolic abnormalities in Williams-Beuren syndrome.

    PubMed

    Palacios-Verdú, María Gabriela; Segura-Puimedon, Maria; Borralleras, Cristina; Flores, Raquel; Del Campo, Miguel; Campuzano, Victoria; Pérez-Jurado, Luis Alberto

    2015-04-01

    Williams-Beuren syndrome (WBS, OMIM-194050) is a neurodevelopmental disorder with multisystemic manifestations caused by a 1.55-1.83 Mb deletion at 7q11.23 including 26-28 genes. Reported endocrine and metabolic abnormalities include transient hypercalcaemia of infancy, subclinical hypothyroidism in ∼ 30% of children and impaired glucose tolerance in ∼ 75% of adult individuals. The purpose of this study was to further study metabolic alterations in patients with WBS, as well as in several mouse models, to establish potential candidate genes. We analysed several metabolic parameters in a cohort of 154 individuals with WBS (data available from 69 to 151 cases per parameter), as well as in several mouse models with complete and partial deletions of the orthologous WBS locus, and searched for causative genes and potential modifiers. Triglyceride plasma levels were significantly decreased in individuals with WBS while cholesterol levels were slightly decreased compared with controls. Hyperbilirubinemia, mostly unconjugated, was found in 18.3% of WBS cases and correlated with subclinical hypothyroidism and hypotriglyceridemia, suggesting common pathogenic mechanisms. Haploinsufficiency at MLXIPL and increased penetrance for hypomorphic alleles at the UGT1A1 gene promoter might underlie the lipid and bilirubin alterations. Other disturbances included increased protein and iron levels, as well as the known subclinical hypothyroidism and glucose intolerance. Our results show that several unreported biochemical alterations, related to haploinsufficiency for specific genes at 7q11.23, are relatively common in WBS. The early diagnosis, follow-up and management of these metabolic disturbances could prevent long-term complications in this disorder. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  9. 77 FR 76414 - William D. Ford Federal Direct Loan Program

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-12-28

    ... DEPARTMENT OF EDUCATION 34 CFR Part 685 RIN 1840-AC94 [Docket ID ED-2008-OPE-0009] William D. Ford... Loan (Perkins Loan) Program; the Federal Family Education Loan (FFEL) Program; and the William D. Ford.... Accordingly, 34 CFR part 685 is corrected as follows: PART 685--WILLIAM D. FORD FEDERAL DIRECT LOAN PROGRAM 0...

  10. Mental Health Problems in Adults with Williams Syndrome

    ERIC Educational Resources Information Center

    Stinton, Chris; Elison, Sarah; Howlin, Patricia

    2010-01-01

    Although many researchers have investigated emotional and behavioral difficulties in individuals with Williams syndrome, few have used standardized diagnostic assessments. We examined mental health problems in 92 adults with Williams syndrome using the Psychiatric Assessment Schedule for Adults with Developmental Disabilities--PAS-ADD (Moss,…

  11. William Burke Critchfield

    Treesearch

    Connie Millar

    1990-01-01

    William B. Critchfield died July 11, 1989. He left a legacy unparalleled in forest genetics. Bill made major contributions to understanding genetic variation, hybridization, growth and development, biogeography, paleobotany, systematics, and taxonomy of forest trees, especially pines. In each of these fields, Bill's pioneering work earned him the position of world...

  12. 34 CFR 685.100 - The William D. Ford Federal Direct Loan Program.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 34 Education 3 2010-07-01 2010-07-01 false The William D. Ford Federal Direct Loan Program. 685...) OFFICE OF POSTSECONDARY EDUCATION, DEPARTMENT OF EDUCATION WILLIAM D. FORD FEDERAL DIRECT LOAN PROGRAM Purpose and Scope § 685.100 The William D. Ford Federal Direct Loan Program. (a) Under the William D. Ford...

  13. Recent paleoseismicity record in Prince William Sound, Alaska, USA

    NASA Astrophysics Data System (ADS)

    Kuehl, Steven A.; Miller, Eric J.; Marshall, Nicole R.; Dellapenna, Timothy M.

    2017-12-01

    Sedimentological and geochemical investigation of sediment cores collected in the deep (>400 m) central basin of Prince William Sound, along with geochemical fingerprinting of sediment source areas, are used to identify earthquake-generated sediment gravity flows. Prince William Sound receives sediment from two distinct sources: from offshore (primarily Copper River) through Hinchinbrook Inlet, and from sources within the Sound (primarily Columbia Glacier). These sources are found to have diagnostic elemental ratios indicative of provenance; Copper River Basin sediments were significantly higher in Sr/Pb and Cu/Pb, whereas Prince William Sound sediments were significantly higher in K/Ca and Rb/Sr. Within the past century, sediment gravity flows deposited within the deep central channel of Prince William Sound have robust geochemical (provenance) signatures that can be correlated with known moderate to large earthquakes in the region. Given the thick Holocene sequence in the Sound ( 200 m) and correspondingly high sedimentation rates (>1 cm year-1), this relationship suggests that sediments within the central basin of Prince William Sound may contain an extraordinary high-resolution record of paleoseismicity in the region.

  14. Emotional Responsivity in Young Children with Williams Syndrome

    ERIC Educational Resources Information Center

    Fidler, Debbie J.; Hepburn, Susan L.; Most, David E.; Philofsky, Amy; Rogers, Sally J.

    2007-01-01

    The hypothesis that young children with Williams syndrome show higher rates of emotional responsivity relative to other children with developmental disabilities was explored. Performance of 23 young children with Williams syndrome and 30 MA-matched children with developmental disabilities of nonspecific etiologies was compared on an adaptation of…

  15. Osler usque ad mare: the SS William Osler

    PubMed Central

    Bryan, C S; Fransiszyn, M

    1999-01-01

    William Osler's connections with the sea included a strong family history of seafaring, his own transatlantic crossings (of which there were at least 32) and the occasional use of nautical imagery in his inspirational writings. An unusual Oslerian connection with the sea emerged after his death in the form of a World War II Liberty ship. Through the SS William Osler and its sister ships, Osler was symbolically reunited with colleagues associated with the early days of the Johns Hopkins Hospital. The William Osler circumnavigated the globe in 1943 without engaging the enemy. She was then converted into an army hospital ship and renamed the USHS Wisteria. PMID:10530306

  16. Infantile ictal apneas in a child with williams-beuren syndrome.

    PubMed

    Myers, Kenneth A; McLeod, D Ross; Bello-Espinosa, Luis

    2013-02-01

    Williams-Beuren syndrome is a genetic disorder rarely associated with seizures. The few described cases of Williams-Beuren syndrome and epilepsy have primarily involved infantile spasms and deletions extending beyond the common deletion region for this disorder. We present the case of a 5-week-old child with ictal apneas and typical Williams-Beuren syndrome deletion. Diagnosis was challenging, because the child had cardiac, respiratory, and gastrointestinal abnormalities typically associated with Williams-Beuren syndrome, which are also associated with cyanotic episodes. The results of interictal electroencephalography were normal, illustrating that prolonged electroencephalography is often essential in evaluation of suspected ictal apneas. Seizure freedom was achieved with carbamazepine. Sudden death is seen in Williams-Beuren syndrome, and this case raises the question whether some of these cases may be related to ictal apneas and could potentially be preventable with appropriate pharmaceutical intervention. Copyright © 2013 Elsevier Inc. All rights reserved.

  17. Celiac disease in patients with Williams-Beuren syndrome.

    PubMed

    Mıhçı, Ercan; Nur, Banu Güzel; Berker-Karaüzüm, Sibel; Yılmaz, Aygen; Artan, Reha

    2015-01-01

    Celiac disease is an autoimmune, gastrointestinal disorder characterized by intolerance to the dietary grain protein gluten. An increased prevalence of celiac disease has been reported in Down syndrome and Turner syndrome, but there has been only few previous reports with respect to the association of celiac disease in Williams-Beuren syndrome. The aim of this study was to evaluate the frequency of celiac disease in our 24 Williams-Beuren syndrome patients. Gastrointestinal problems and celiac disease symptoms of patients were noted. All patients were analyzed by the titer of tissue transglutaminases IgA and IgG. HLA genotyping and intestinal biopsy was performed to the patients with positive serology. We also performed gluten free diet in the presence of compatible symptoms, serology, HLA genotyping and intestinal biopsy. In our study, two patients had positive tTG antibodies, but only one had positive biopsy finding for celiac disease. The frequency of celiac disease in patients with Williams-Beuren syndrome was estimated as 1/24 (4.1%). Though the number of participants in this study was limited, the results show that the frequency of celiac disease is higher in Williams-Beuren syndrome compared to the general population. We suggest that a high suspicion and testing for celiac disease should be recommended at certain intervals in all cases with Williams-Beuren syndrome to detect the cause of growth retardation and gastrointestinal problems.

  18. The circulation of Prince William Sound

    NASA Technical Reports Server (NTRS)

    Muench, R. D. (Principal Investigator)

    1973-01-01

    The author has identified the following significant results. Results suggest that sediment-laden plumes of fresh water from rivers may be useful tracers, due to their high visibility, of surface water motion. The two useable images obtained to date corroborate that westerly flow was occurring in the Gulf of Alaska just south of Prince William Sound, and that an inflow into Prince William Sound was occurring concurrently with flood tides on both occasions. River plumes are useful tracers, but poor weather conditions somewhat limit the use of satellite imagery.

  19. The Williams Review

    ERIC Educational Resources Information Center

    Knight, Rupert

    2009-01-01

    The publication in June of Sir Peter Williams' review of mathematics teaching in early years' settings and primary schools came at a time when the thoughts of many teachers were turning towards a well-earned summer break. However, the report has attracted much attention and promises a fundamental shake-up of attitudes and approaches to the…

  20. Haploinsufficiency of BAZ1B contributes to Williams syndrome through transcriptional dysregulation of neurodevelopmental pathways.

    PubMed

    Lalli, Matthew A; Jang, Jiwon; Park, Joo-Hye C; Wang, Yidi; Guzman, Elmer; Zhou, Hongjun; Audouard, Morgane; Bridges, Daniel; Tovar, Kenneth R; Papuc, Sorina M; Tutulan-Cunita, Andreea C; Huang, Yadong; Budisteanu, Magdalena; Arghir, Aurora; Kosik, Kenneth S

    2016-04-01

    Williams syndrome (WS) is a neurodevelopmental disorder caused by a genomic deletion of ∼28 genes that results in a cognitive and behavioral profile marked by overall intellectual impairment with relative strength in expressive language and hypersocial behavior. Advancements in protocols for neuron differentiation from induced pluripotent stem cells allowed us to elucidate the molecular circuitry underpinning the ontogeny of WS. In patient-derived stem cells and neurons, we determined the expression profile of the Williams-Beuren syndrome critical region-deleted genes and the genome-wide transcriptional consequences of the hemizygous genomic microdeletion at chromosome 7q11.23. Derived neurons displayed disease-relevant hallmarks and indicated novel aberrant pathways in WS neurons including over-activated Wnt signaling accompanying an incomplete neurogenic commitment. We show that haploinsufficiency of the ATP-dependent chromatin remodeler, BAZ1B, which is deleted in WS, significantly contributes to this differentiation defect. Chromatin-immunoprecipitation (ChIP-seq) revealed BAZ1B target gene functions are enriched for neurogenesis, neuron differentiation and disease-relevant phenotypes. BAZ1B haploinsufficiency caused widespread gene expression changes in neural progenitor cells, and together with BAZ1B ChIP-seq target genes, explained 42% of the transcriptional dysregulation in WS neurons. BAZ1B contributes to regulating the balance between neural precursor self-renewal and differentiation and the differentiation defect caused by BAZ1B haploinsufficiency can be rescued by mitigating over-active Wnt signaling in neural stem cells. Altogether, these results reveal a pivotal role for BAZ1B in neurodevelopment and implicate its haploinsufficiency as a likely contributor to the neurological phenotypes in WS. © The Author 2016. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  1. 76 FR 44893 - Prince William Sound Resource Advisory Committee

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-07-27

    ... DEPARTMENT OF AGRICULTURE Forest Service Prince William Sound Resource Advisory Committee AGENCY: Forest Service, USDA. ACTION: Notice of meeting. SUMMARY: The Prince William Sound Resource Advisory... District, 145 Forest Station Road, Girdwood, AK; Prince Willam Sound Community College, 303 Lowe Street...

  2. 33 CFR 165.1704 - Prince William Sound, Alaska-regulated navigation area.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 33 Navigation and Navigable Waters 2 2013-07-01 2013-07-01 false Prince William Sound, Alaska... District § 165.1704 Prince William Sound, Alaska-regulated navigation area. (a) The following is a... Hinchinbrook Light to Schooner Rock Light, comprising that portion of Prince William Sound between 146°30′ W...

  3. 33 CFR 165.1704 - Prince William Sound, Alaska-regulated navigation area.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 33 Navigation and Navigable Waters 2 2012-07-01 2012-07-01 false Prince William Sound, Alaska... District § 165.1704 Prince William Sound, Alaska-regulated navigation area. (a) The following is a... Hinchinbrook Light to Schooner Rock Light, comprising that portion of Prince William Sound between 146°30′ W...

  4. 33 CFR 165.1704 - Prince William Sound, Alaska-regulated navigation area.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 33 Navigation and Navigable Waters 2 2014-07-01 2014-07-01 false Prince William Sound, Alaska... District § 165.1704 Prince William Sound, Alaska-regulated navigation area. (a) The following is a... Hinchinbrook Light to Schooner Rock Light, comprising that portion of Prince William Sound between 146°30′ W...

  5. 33 CFR 165.1704 - Prince William Sound, Alaska-regulated navigation area.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 33 Navigation and Navigable Waters 2 2010-07-01 2010-07-01 false Prince William Sound, Alaska... District § 165.1704 Prince William Sound, Alaska-regulated navigation area. (a) The following is a... Hinchinbrook Light to Schooner Rock Light, comprising that portion of Prince William Sound between 146°30′ W...

  6. 33 CFR 165.1704 - Prince William Sound, Alaska-regulated navigation area.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 33 Navigation and Navigable Waters 2 2011-07-01 2011-07-01 false Prince William Sound, Alaska... District § 165.1704 Prince William Sound, Alaska-regulated navigation area. (a) The following is a... Hinchinbrook Light to Schooner Rock Light, comprising that portion of Prince William Sound between 146°30′ W...

  7. Mailability v. the Crusader: Williams v. O'Brien.

    ERIC Educational Resources Information Center

    Simmons, Charles E.

    The issues of prior restraint and press censorship are examined in this paper, which focuses on the 1970 Williams v. O'Brien court case. The paper discusses the litigation, in which Robert F. Williams, as an American citizen living in Peking, China, sued the United States Postmaster General over the banning of the May 1967 issue of "The…

  8. Prince William Forest Park American Beech , Approximately one mile ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    Prince William Forest Park American Beech , Approximately one mile from visitor’s center, south bank of the south fork of Quantico Creek, about 75 yards upstream from its confluence with Quantico Creek, Near Birch Bluff Trail, Triangle, Prince William County, VA

  9. Assessment of clinical scoring systems for the diagnosis of Williams-Beuren syndrome.

    PubMed

    Leme, D E S; Souza, D H; Mercado, G; Pastene, E; Dias, A; Moretti-Ferreira, D

    2013-09-04

    Williams-Beuren syndrome (WBS) is a genetic disorder characterized by physical and intellectual developmental delay, associated with congenital heart disease and facial dysmorphism. WBS is caused by a microdeletion on chromosome 7 (7q11.23), which encompasses the elastin (ELN) gene and about 27 other genes. The gold standard for WBS laboratory diagnosis is FISH (fluorescence in situ hybridization), which is very costly. As a possible alternative, we investigated the accuracy of three clinical diagnostic scoring systems in 250 patients with WBS diagnosed by FISH. We concluded that all three systems could be used for the clinical diagnosis of WBS, but they all gave a low percentage of false-positive (6.0-9.2%) and false-negative (0.8-4.0%) results. Therefore, their use should be associated with FISH testing.

  10. 77 FR 19301 - Prince William Sound Regional Citizens' Advisory Council Charter Renewal

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-03-30

    ... DEPARTMENT OF HOMELAND SECURITY Coast Guard [USCG-2012-0099] Prince William Sound Regional... Prince William Sound Regional Citizens' Advisory Council (PWSRCAC) as an alternative voluntary advisory group for Prince William Sound, Alaska. This certification allows the PWSRCAC to monitor the activities...

  11. Interaction with William Carnall

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Judd, Brian R.

    2005-02-15

    A personal account is given of interaction with William T. Carnall during the period 1977-1988, when I made regular visits to the Argonne National Laboratory to discuss the theoretical background to the spectroscopic work he was carrying out on the lanthanides and actinides.

  12. Williams Exercises in Zvezda

    NASA Image and Video Library

    2007-01-13

    ISS014-E-11786 (13 Jan. 2007) --- Surrounded by hardware, astronaut Sunita L. Williams, Expedition 14 flight engineer, equipped with a bungee harness, exercises on the Treadmill Vibration Isolation System (TVIS) (out of frame) in the Zvezda Service Module of the International Space Station.

  13. Williams Exercises in Zvezda

    NASA Image and Video Library

    2007-01-13

    ISS014-E-11784 (13 Jan. 2007) --- Surrounded by hardware, astronaut Sunita L. Williams, Expedition 14 flight engineer, equipped with a bungee harness, exercises on the Treadmill Vibration Isolation System (TVIS) (out of frame) in the Zvezda Service Module of the International Space Station.

  14. Global and local music perception in children with Williams syndrome.

    PubMed

    Deruelle, Christine; Schön, Daniele; Rondan, Cécilie; Mancini, Josette

    2005-04-25

    Musical processing can be decomposed into the appreciation of global and local elements. This global/local dissociation was investigated with the processing of contour-violated and interval-violated melodies. Performance of a group of 16 children with Williams syndrome and a group of 16 control children were compared in a same-different task. Control participants were more accurate in detecting differences in the contour-violated than in the interval-violated condition while Williams syndrome individuals performed equally well in both conditions. This finding suggests that global precedence may occur at an early perceptual stage in normally developing children. In contrast, no such global precedence is observed in the Williams syndrome population. These data are discussed in the context of atypical cognitive profiles of individuals with Williams syndrome.

  15. Behavioral and Emotional Disturbance in Individuals with Williams Syndrome.

    ERIC Educational Resources Information Center

    Einfeld, Stewart L.; Tonge, Bruce J.; Florio, Tony

    1997-01-01

    Comparison of behavioral and emotional disturbance in 70 children and adolescents with Williams Syndrome (characterized by mental retardation and short stature) and a control group, found Williams Syndrome subjects were more likely to be diagnosed with a psychiatric disorder characterized by anxiety, hyperactivity, preoccupations, and…

  16. Constitutional and somatic deletions of the Williams-Beuren syndrome critical region in non-Hodgkin lymphoma.

    PubMed

    Guenat, David; Quentin, Samuel; Rizzari, Carmelo; Lundin, Catarina; Coliva, Tiziana; Edery, Patrick; Fryssira, Helen; Bermont, Laurent; Ferrand, Christophe; Soulier, Jean; Borg, Christophe; Rohrlich, Pierre-Simon

    2014-11-07

    Here, we report and investigate the genomic alterations of two novel cases of Non-Hodgkin Lymphoma (NHL) in children with Williams-Beuren syndrome (WBS), a multisystem disorder caused by 7q11.23 hemizygous deletion. Additionally, we report the case of a child with NHL and a somatic 7q11.23 deletion. Although the WBS critical region has not yet been identified as a susceptibility locus in NHL, it harbors a number of genes involved in DNA repair. The high proportion of pediatric NHL reported in WBS is intriguing. Therefore, the role of haploinsufficiency of genes located at 7q11.23 in lymphomagenesis deserves to be investigated.

  17. 2. WILLIAM ELLIOT CABIN AND OUTBULIDING, CABIN WEST REAR AND ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    2. WILLIAM ELLIOT CABIN AND OUTBULIDING, CABIN WEST REAR AND NORTH SIDES, OUTBULIDING WEST FRONT AND NORTH SIDE - Liberty Historic District, William Elliot Cabin, Route 2, Cle Elum, Liberty, Kittitas County, WA

  18. 1. WILLIAM ELLIOT CABIN AND OUTBUILDING, CABIN EAST FRONT AND ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    1. WILLIAM ELLIOT CABIN AND OUTBUILDING, CABIN EAST FRONT AND SOUTH SIDE, OUTBUILDING EAST REAR AND SOUTH SIDES - Liberty Historic District, William Elliot Cabin, Route 2, Cle Elum, Liberty, Kittitas County, WA

  19. [Association between Williams syndrome and adrenal insufficiency].

    PubMed

    Rchachi, Meryem; Larwanou, Maazou Mahamane; El Ouahabi, Hanan; Ajdi, Farida

    2017-01-01

    Williams syndrome is a developmental disorder including dysmorphia, cardiovascular malformations and a specific neuropsychological profile together with other associated disorders. We report the case of a 17-year old girl, born of a non-inbred marriage, with Williams syndrome discovered during an assessment of degree of failure to thrive. Its association with primary adrenal insufficiency makes it unique. Diagnosis is confirmed by cytogenetic and molecular analysis. Its management consists of the implementation of treatment for adrenal insufficiency associated with a clinico-biological monitoring.

  20. White matter integrity deficits in prefrontal-amygdala pathways in Williams syndrome.

    PubMed

    Avery, Suzanne N; Thornton-Wells, Tricia A; Anderson, Adam W; Blackford, Jennifer Urbano

    2012-01-16

    Williams syndrome is a neurodevelopmental disorder associated with significant non-social fears. Consistent with this elevated non-social fear, individuals with Williams syndrome have an abnormally elevated amygdala response when viewing threatening non-social stimuli. In typically-developing individuals, amygdala activity is inhibited through dense, reciprocal white matter connections with the prefrontal cortex. Neuroimaging studies suggest a functional uncoupling of normal prefrontal-amygdala inhibition in individuals with Williams syndrome, which might underlie both the extreme amygdala activity and non-social fears. This functional uncoupling might be caused by structural deficits in underlying white matter pathways; however, prefrontal-amygdala white matter deficits have yet to be explored in Williams syndrome. We used diffusion tensor imaging to investigate prefrontal-amygdala white matter integrity differences in individuals with Williams syndrome and typically-developing controls with high levels of non-social fear. White matter pathways between the amygdala and several prefrontal regions were isolated using probabilistic tractography. Within each pathway, we tested for between-group differences in three measures of white matter integrity: fractional anisotropy (FA), radial diffusivity (RD), and parallel diffusivity (λ(1)). Individuals with Williams syndrome had lower FA, compared to controls, in several of the prefrontal-amygdala pathways investigated, indicating a reduction in white matter integrity. Lower FA in Williams syndrome was explained by significantly higher RD, with no differences in λ(1), suggestive of lower fiber density or axon myelination in prefrontal-amygdala pathways. These results suggest that deficits in the structural integrity of prefrontal-amygdala white matter pathways might underlie the increased amygdala activity and extreme non-social fears observed in Williams syndrome. Copyright © 2011 Elsevier Inc. All rights reserved.

  1. 76 FR 2902 - Williams, Barry Lawson; Notice of Filing

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-01-18

    ... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Docket No. ID-2449-005] Williams, Barry Lawson; Notice of Filing January 10, 2011. Take notice that on January 10, 2011, Barry Lawson Williams... [email protected] , or call (866) 208-3676 (toll free). For TTY, call (202) 502-8659. Comment Date...

  2. 75 FR 62530 - Williams, Barry Lawson; Notice of Filing

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-10-12

    ... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Docket No. ID-6409-000] Williams, Barry Lawson; Notice of Filing October 4, 2010. Take notice that on September 24, 2010, Barry Lawson Williams..., please e-mail [email protected] , or call (866) 208-3676 (toll free). For TTY, call (202) 502...

  3. Genetics Home Reference: Williams syndrome

    MedlinePlus

    ... Berman KF. Neural correlates of genetically abnormal social cognition in Williams syndrome. Nat Neurosci. 2005 Aug;8( ... syndrome: a unique window to genetic influences on cognition and behaviour. Nat Rev Neurosci. 2006 May;7( ...

  4. SLEEP - Williams wearing sleep net

    NASA Image and Video Library

    1998-05-12

    STS090-377-011 (17 APRIL-3 MAY 1998) --- Astronaut Dafydd R. (Dave) Williams, mission specialist representing the Canadian Space Agency (CSA), accomplishes more than one purpose when he sleeps in this bunk aboard the Earth-orbiting Space Shuttle Columbia. Conducting a Neurolab sleep experiment, Williams wears equipment which includes a sleep net (mesh cap that monitors and records brain waves); a Respiratory Inductance Plethysmograph (RIP) suit for monitoring respiration; and an activity monitor -- a device (out of view) worn on the wrist to detect and record body movement. Data on brain waves, eye movements, respiration, heart rate, and oxygen concentration are routed to a portable data recorder. The entire system has capabilities similar to a fully equipped sleep laboratory on Earth. The sleeping bag is conventional Shuttle ware and not part of the experiment.

  5. View of Mission Street facing south west. Williams Building is ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    View of Mission Street facing south west. Williams Building is at left background, Aron (Mercantile) Building is at right background. Parking lot is in left foreground - Williams Building, 693 Mission Street, San Francisco, San Francisco County, CA

  6. Williams in US Lab

    NASA Image and Video Library

    2010-02-10

    S130-E-006844 (10 Feb. 2010) --- NASA astronaut Jeffrey Williams, Expedition 22 commander, installs a Urine Processor Assembly / Distillation Assembly (UPA DA) in the Water Recovery System (WRS) rack in the Destiny laboratory of the International Space Station while space shuttle Endeavour (STS-130) remains docked with the station.

  7. Profiles in drug metabolism and toxicology: Richard Tecwyn Williams (1909-1979).

    PubMed

    Jones, Alan Wayne

    2015-01-01

    This article pays homage to the life and work of a veritable pioneer in toxicology and drug metabolism, namely a Welshman, Richard Tecwyn Williams, FRS. Professor Williams, or RT as he was known, made major contributions to knowledge about the metabolism and toxicology of drugs and xenobiotics during a scientific career spanning nearly 50 years. Author or coauthor of close to 400 research articles and reviews, including a classic book, entitled Detoxication Mechanisms, Williams and his research school investigated virtually all aspects of drug metabolism, especially conjugations. In particular, the concepts of phase 1 and phase II metabolic pathways were introduced by Williams; the biliary excretion of drugs was extensively studied as were species differences in drug metabolism and detoxication. Besides investigating the metabolism of many pharmaceutical drugs, such as sulfonamides and thalidomide, Williams and his group investigated the disposition and fate in the body of organic pesticides and recreational drugs of abuse, such as amphetamine, methamphetamine and lysergic acid diethylamide (LSD).

  8. Discovering Structure in Auditory Input: Evidence from Williams Syndrome

    ERIC Educational Resources Information Center

    Elsabbagh, Mayada; Cohen, Henri; Karmiloff-Smith, Annette

    2010-01-01

    We examined auditory perception in Williams syndrome by investigating strategies used in organizing sound patterns into coherent units. In Experiment 1, we investigated the streaming of sound sequences into perceptual units, on the basis of pitch cues, in a group of children and adults with Williams syndrome compared to typical controls. We showed…

  9. 75 FR 16159 - Prince William Sound Regional Citizens' Advisory Council (PWSRCAC) Charter Renewal

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-03-31

    ... DEPARTMENT OF HOMELAND SECURITY Coast Guard [USCG-2010-0121] Prince William Sound Regional... the Prince William Sound Regional Citizens' Advisory Council (PWSRCAC) as an alternative voluntary advisory group for Prince William Sound, Alaska. This certification allows the PWSRCAC to monitor the...

  10. William Wilde and 1 Merrion Square.

    PubMed

    McEntegart, R

    2016-05-01

    William Wilde spent the final third of his life, from 1855 to 1876, in 1 Merrion Square. During the first half of his occupancy of the house his career blossomed to its fullest; the second decade, on the other hand, was marked by scandal, personal tragedy, and an unhappy professional and social decline. This paper considers the background to the development of Merrion Square, the architectural history of 1 Merrion Square from its building in 1762 to the arrival of the Wildes in 1855, the attractions and possibilities which the house offered for William Wilde, the major architectural expansion of the building which he commissioned in 1859, and aspects of his and his family's life in the house.

  11. 78 FR 18616 - Prince William Sound Regional Citizens' Advisory Council (PWSRCAC) Charter Renewal

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-03-27

    ... DEPARTMENT OF HOMELAND SECURITY Coast Guard [Docket No. USCG-2013-0088] Prince William Sound... the Prince William Sound Regional Citizens' Advisory Council (PWSRCAC) as an alternative voluntary advisory group for Prince William Sound, Alaska. This certification allows the PWSRCAC to monitor the...

  12. 76 FR 24506 - Prince William Sound Regional Citizens' Advisory Council (PWSRCAC) Charter Renewal

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-05-02

    ... DEPARTMENT OF HOMELAND SECURITY Coast Guard [Docket No. USCG-2011-0142] Prince William Sound... the Prince William Sound Regional Citizens' Advisory Council (PWSRCAC) as an alternative voluntary advisory group for Prince William Sound, Alaska. This certification allows the PWSRCAC to monitor the...

  13. Earthquake Signatures in the Modern Sediment Record of Prince William Sound, Alaska

    NASA Astrophysics Data System (ADS)

    Marshall, N. R.; Kuehl, S. A.; Dellapenna, T. M.; Miller, E. J.

    2016-02-01

    Geochemical signatures of earthquake-generated sediment gravity flows are investigated using X-ray fluorescence core scanning on a suite of sediment cores from Prince William Sound, Alaska. This study focused on the development of geochemical proxies for earthquake deposits with an emphasis on interpreting deposits initiated from large subduction earthquakes. A north-south transect of sediment cores from Prince William Sound, between Hinchinbrook Island and the Columbia Glacier, was used to examine a record of earthquakes in this tectonically active region for the past century. The sediments in Prince William Sound are sourced from two geologically distinct regions: the metamorphosed turbidites of coastal Prince William Sound, and the Copper River Basin that contains a significant amount of volcanic rocks. Geochemical studies of sediment cores and end-member sediment samples using X-ray fluorescence and inductively coupled plasma mass spectrometry allowed for the development of geochemical proxies for sediment provenance during the past 100 years. Downcore peaks in Sr/Pb are indicative of Copper River Basin sediments, whereas peaks in K/Ca are indicative of inputs of Prince William Sound sediments. Large subduction earthquakes in northern Prince William Sound initiate gravity flows of Prince William Sound provenance into the deep channel. Particularly robust provenance signatures are seen in the northernmost cores in the core transect, which are closer to the earthquake epicenters and the Columbia Glacier source region. The ages of the deposits, from core-averaged 210Pb sediment accumulation rates, correspond to large earthquakes that occurred in 1912, 1964, and 1983. A similar deposit from 1895 in northern Prince William Sound, prior to historical earthquake records, may have also been initiated from a large earthquake in the 1890's.

  14. Williams cuts his hair in the SM during Expedition 13

    NASA Image and Video Library

    2006-05-09

    ISS013-E-17184 (9 May 2006) --- Astronaut Jeffrey N. Williams, Expedition 13 NASA space station science officer and flight engineer, trims his hair in the Zarya module of the International Space Station. Williams used hair clippers fashioned with a vacuum device to garner freshly cut hair.

  15. Williams-Beuren syndrome: phenotypic variability and deletions of chromosomes 7, 11, and 22 in a series of 52 patients.

    PubMed Central

    Joyce, C A; Zorich, B; Pike, S J; Barber, J C; Dennis, N R

    1996-01-01

    Fluorescence in situ hybridisation (FISH) and conventional chromosome analysis were performed on a series of 52 patients with classical Williams-Beuren syndrome (WBS), suspected WBS, or supravalvular aortic stenosis (SVAS). In the classical WBS group, 22/23 (96%) had a submicroscopic deletion of the elastin locus on chromosome 7, but the remaining patient had a unique interstitial deletion of chromosome 11 (del(11)(q13.5q14.2)). In the suspected WBS group 2/22 (9%) patients had elastin deletions but a third patient had a complex karyotype including a ring chromosome 22 with a deletion of the long arm (r(22)(p11-->q13)). In the SVAS group, 1/7 (14%) had an elastin gene deletion, despite having normal development and minimal signs of WBS. Overall, some patients with submicroscopic elastin deletions have fewer features of Williams-Beuren syndrome than those with other cytogenetic abnormalities. These results, therefore, emphasise the importance of a combined conventional and molecular cytogenetic approach to diagnosis and suggest that the degree to which submicroscopic deletions of chromosome 7 extend beyond the elastin locus may explain some of the phenotypic variability found in Williams-Beuren syndrome. Images PMID:9004128

  16. The use of emotions in narratives in Williams syndrome.

    PubMed

    Van Herwegen, Jo; Aznar, Ana; Tenenbaum, Harriet

    2014-01-01

    Although individuals with Williams syndrome are very sociable, they tend to have limited contact and friendships with peers. In typically developing children the use of positive emotions (e.g., happy) has been argued to be related to peer relationships and popularity. The current study investigated the use and development of emotion words in Williams syndrome using cross-sectional developmental trajectories and examined children's use of different types of emotion words. Nineteen children with Williams syndrome (WS) and 20 typically developing (TD) children matched for chronological age told a story from a wordless picture book. Participants with WS produced a similar number of emotion words compared to the control group and the use of emotion words did not change when plotted against chronological age or vocabulary abilities in either group. However, participants with WS produced more emotion words about sadness. Links between emotion production and friendships as well as future studies are discussed. After reading this article, readers will be able to: explain the development of positive and negative emotions in Williams syndrome and recognize that emotion production is atypical in this population. Copyright © 2014 Elsevier Inc. All rights reserved.

  17. Probed Serial Recall in Williams Syndrome: Lexical Influences on Phonological Short-Term Memory

    ERIC Educational Resources Information Center

    Brock, Jan; McCormack, Teresa; Boucher, Jill

    2005-01-01

    Williams syndrome is a genetic disorder that, it has been claimed, results in an unusual pattern of linguistic strengths and weaknesses. The current study investigated the hypothesis that there is a reduced influence of lexical knowledge on phonological short-term memory in Williams syndrome. Fourteen children with Williams syndrome and 2…

  18. Pursuing the Panderer: An Analysis of "United States v. Williams"

    ERIC Educational Resources Information Center

    McGrain, Patrick N.; Moore, Jennifer L.

    2010-01-01

    In May 2008, the Supreme Court addressed whether the government can regulate the ownership and distribution of virtual child pornography. "U.S. v. Williams" marked the first time the Court directly addressed the concept of pandering virtual child pornography. This article examines the Court's decision in "U.S. v. Williams" and…

  19. Unveiling of sign for Walter C. Williams Research Aircraft Integration Facility

    NASA Technical Reports Server (NTRS)

    1995-01-01

    In a brief ceremony following a memorial service for the late Walter C. Williams on November 17, 1995, the Integrated Test Facility (ITF) at the NASA Dryden Flight Research Center at Edwards, California, was formally renamed the Walter C. Williams Research Aircraft Integration Facility. Shown is the family of Walt Williams: Helen, his widow, sons Charles and Howard, daughter Elizabeth Williams Powell, their spouses and children unveiling the new sign redesignating the Facility. The test facility provides state-of-the-art capabilities for thorough ground testing of advanced research aircraft. It allows researchers and technicians to integrate and test aircraft systems before each research flight, which greatly enhances the safety of each mission. In September 1946 Williams became engineer-in-charge of a team of five engineers who arrived at Muroc Army Air Base (now Edwards AFB) from the National Advisory Committee for Aeronautics's Langley Memorial Aeronautical Laboratory, Hampton, Virginia (now NASA's Langley Research Center), to prepare for supersonic research flights in a joint NACA-Army Air Forces program involving the rocket-powered X-1. This established the first permanent NACA presence at the Mojave Desert site although initially the five engineers and others who followed them were on temporary assignment. Over time, Walt continued to be in charge during the many name changes for the NACA-NASA organization, with Williams ending his stay as Chief of the NASA Flight Research Center in September 1959 (today NASA's Dryden Flight Research Center).

  20. Using novel control groups to dissect the amygdala's role in Williams syndrome.

    PubMed

    Thornton-Wells, Tricia A; Avery, Suzanne N; Blackford, Jennifer Urbano

    2011-07-01

    Williams syndrome is a neurodevelopmental disorder with an intriguing behavioral phenotype-hypersociability combined with significant non-social fears. Previous studies have demonstrated abnormalities in amygdala function in individuals with Williams syndrome compared to typically-developing controls. However, it remains unclear whether the findings are related to the atypical neurodevelopment of Williams syndrome, or are also associated with behavioral traits at the extreme end of a normal continuum. We used functional magnetic resonance imaging (fMRI) to compare amygdala blood-oxygenation-level-dependent (BOLD) responses to non-social and social images in individuals with Williams syndrome compared to either individuals with inhibited temperament (high non-social fear) or individuals with uninhibited temperament (high sociability). Individuals with Williams syndrome had larger amygdala BOLD responses when viewing the non-social fear images than the inhibited temperament control group. In contrast, when viewing both fear and neutral social images, individuals with Williams syndrome did not show smaller amygdala BOLD responses relative to the uninhibited temperament control group, but instead had amygdala responses proportionate to their sociability. These results suggest heightened amygdala response to non-social fear images is characteristic of WS, whereas, variability in amygdala response to social fear images is proportionate to, and might be explained by, levels of trait sociability.

  1. William J. Glackens: "The Cedar Walk."

    ERIC Educational Resources Information Center

    Davidson, Marilyn

    1986-01-01

    Provides a lesson plan for primary grade-level students based on William J. Glackens' oil painting, "The Cedar Walk." The goal of the lesson is to introduce students to landscape/seascape painting. (JDH)

  2. 76 FR 1187 - Application for Recertification of Prince William Sound Regional Citizens' Advisory Council

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-01-07

    ... Prince William Sound Regional Citizens' Advisory Council AGENCY: Coast Guard, DHS. ACTION: Notice of... on, the application for recertification submitted by the Prince William Sound Regional Citizen's... advisory group in lieu of a Regional Citizens' Advisory Council for Prince William Sound, Alaska. This...

  3. FE Williams exercising on the CEVIS

    NASA Image and Video Library

    2012-07-21

    ISS032-E-008595 (20 July 2012) --- NASA astronaut Sunita Williams, Expedition 32 flight engineer, exercises on the Cycle Ergometer with Vibration Isolation System (CEVIS) in the Destiny laboratory of the International Space Station.

  4. John Dalton and the London atomists: William and Bryan Higgins, William Austin, and new Daltonian doubts about the origin of the atomic theory

    PubMed Central

    Grossman, Mark I.

    2014-01-01

    Most historians have ruled out the possibility that John Dalton was influenced by the theories of atomists William and Bryan Higgins, as well as William Austin, in developing his first table of atomic weights on 6 September 1803. I review and evaluate the case to be made for the influence of each scientist on Dalton. Contrary to prevailing views, I raise new Daltonian doubts, especially for Bryan Higgins.

  5. Williams performs IFM during Expedition 13

    NASA Image and Video Library

    2006-08-24

    ISS013-E-70896 (24 Aug. 2006) --- Astronaut Jeffrey N. Williams, Expedition 13 NASA space station science officer and flight engineer, performs in-flight maintenance (IFM) in the Destiny laboratory of the International Space Station.

  6. Williams Syndrome Transcription Factor is critical for neural crest cell function in Xenopus laevis

    PubMed Central

    Barnett, Chris; Yazgan, Oya; Kuo, Hui-Ching; Malakar, Sreepurna; Thomas, Trevor; Fitzgerald, Amanda; Harbour, Billy; Henry, Jonathan J.; Krebs, Jocelyn E.

    2012-01-01

    Williams Syndrome Transcription Factor (WSTF) is one of ~25 haplodeficient genes in patients with the complex developmental disorder Williams Syndrome (WS). WS results in visual/spatial processing defects, cognitive impairment, unique behavioral phenotypes, characteristic “elfin” facial features, low muscle tone and heart defects. WSTF exists in several chromatin remodeling complexes and has roles in transcription, replication, and repair. Chromatin remodeling is essential during embryogenesis, but WSTF’s role in vertebrate development is poorly characterized. To investigate the developmental role of WSTF, we knocked down WSTF in Xenopus laevis embryos using a morpholino that targets WSTF mRNA. BMP4 shows markedly increased and spatially aberrant expression in WSTF-deficient embryos, while SHH, MRF4, PAX2, EPHA4 and SOX2 expression are severely reduced, coupled with defects in a number of developing embryonic structures and organs. WSTF-deficient embryos display defects in anterior neural development. Induction of the neural crest, measured by expression of the neural crest-specific genes SNAIL and SLUG, is unaffected by WSTF depletion. However, at subsequent stages WSTF knockdown results in a severe defect in neural crest migration and/or maintenance. Consistent with a maintenance defect, WSTF knockdowns display a specific pattern of increased apoptosis at the tailbud stage in regions corresponding to the path of cranial neural crest migration. Our work is the first to describe a role for WSTF in proper neural crest function, and suggests that neural crest defects resulting from WSTF haploinsufficiency may be a major contributor to the pathoembryology of WS. PMID:22691402

  7. Tennessee Williams: the uses of declarative memory in The Glass Menagerie.

    PubMed

    Jacobs, Daniel

    2002-01-01

    Tennessee Williams called his first great work, The Glass Menagerie, his "memory play." The situation in which Williams found himself when he began writing the play is explored, as are the ways in which he used the declarative memory of his protagonist, Tom Wingfield, to express and deal with his own painful conflicts. Williams's use of stage directions, lighting, and music to evoke memory and render it three-dimensional is described. Through a close study of The Glass Menagerie, the many uses of memory for the purposes of wish fulfillment, conflict resolution, and resilience are examined.

  8. William Heard Kilpatrick: A Memoir.

    ERIC Educational Resources Information Center

    Van Til, William

    1988-01-01

    A memoir about William Heard Kilpatrick examines his style as a master teacher and leader of educational organizations during the 1930's, providing a personal account of this teacher whose huge classes brought in over $1 million in tuition fees. He promoted progressive education and advocated recognition of individual differences. (SM)

  9. Learning by observation: insights from Williams syndrome.

    PubMed

    Foti, Francesca; Menghini, Deny; Mandolesi, Laura; Federico, Francesca; Vicari, Stefano; Petrosini, Laura

    2013-01-01

    Observing another person performing a complex action accelerates the observer's acquisition of the same action and limits the time-consuming process of learning by trial and error. Observational learning makes an interesting and potentially important topic in the developmental domain, especially when disorders are considered. The implications of studies aimed at clarifying whether and how this form of learning is spared by pathology are manifold. We focused on a specific population with learning and intellectual disabilities, the individuals with Williams syndrome. The performance of twenty-eight individuals with Williams syndrome was compared with that of mental age- and gender-matched thirty-two typically developing children on tasks of learning of a visuo-motor sequence by observation or by trial and error. Regardless of the learning modality, acquiring the correct sequence involved three main phases: a detection phase, in which participants discovered the correct sequence and learned how to perform the task; an exercise phase, in which they reproduced the sequence until performance was error-free; an automatization phase, in which by repeating the error-free sequence they became accurate and speedy. Participants with Williams syndrome beneficiated of observational training (in which they observed an actor detecting the visuo-motor sequence) in the detection phase, while they performed worse than typically developing children in the exercise and automatization phases. Thus, by exploiting competencies learned by observation, individuals with Williams syndrome detected the visuo-motor sequence, putting into action the appropriate procedural strategies. Conversely, their impaired performances in the exercise phases appeared linked to impaired spatial working memory, while their deficits in automatization phases to deficits in processes increasing efficiency and speed of the response. Overall, observational experience was advantageous for acquiring competencies

  10. Valuing Higher Education: An Appreciation of the Work of Gareth Williams

    ERIC Educational Resources Information Center

    Barnett, Ronald, Ed.; Temple, Paul, Ed.; Scott, Peter, Ed.

    2016-01-01

    In "Valuing Higher Education," leading international analysts examine Gareth Williams's contribution to shaping our thinking about the economics of higher education in essays that are a testimony to Williams's conception that the field cannot be properly understood unless viewed alongside social policy, changes in knowledge production,…

  11. Williams exercises on TVIS in Zvezda

    NASA Image and Video Library

    2007-12-28

    ISS014-E-10591 (28 Dec. 2006) --- Astronaut Sunita L. Williams, Expedition 14 flight engineer, equipped with a bungee harness, exercises on the Treadmill Vibration Isolation System (TVIS) in the Zvezda Service Module of the International Space Station.

  12. Williams configures the LMM

    NASA Image and Video Library

    2016-04-18

    ISS047e066551 (04/18/2016) --- NASA astronaut Jeff Williams configures the station’s Light Microscopy Module (LMM), a modified commercial, highly flexible, state-of-the-art light imaging microscope facility that provides researchers with powerful diagnostic hardware and software. The LMM enables novel research of microscopic phenomena in microgravity, with the capability of remotely acquiring and downloading digital images and videos across many levels of magnification.

  13. Williams working on the JAXA MS (Marangoni Surface) Experiment

    NASA Image and Video Library

    2009-11-05

    ISS021-E-020304 (5 Nov. 2009) --- NASA astronaut Jeffrey Williams, Expedition 21 flight engineer, works with Fluid Physics Experiment Facility/Marangoni Surface (FPEF MS) Core hardware in the Kibo laboratory of the International Space Station. Williams first inserted the Marangoni Inside (MI) cassette in the MI Core for a leak check, and then installed the MI Core into the FPEF MI Body. The Marangoni convection experiment in the FPEF examines fluid tension flow in micro-G.

  14. Wilde's worlds: Sir William Wilde in Victorian Ireland.

    PubMed

    McGeachie, J

    2016-05-01

    Other contributors to this collection have evoked the disparate worlds inhabited by Sir William Wilde. To provide an overall assessment of his career. Looking at the historical conditions that made possible such a career spanning such disparate worlds. Deploying methodologies developed by historians of medicine and sociologists of science, the article brings together Wilde the nineteenth century clinician and Dublin man of science, the Wilde of the Census and of the west of Ireland, William Wilde Victorian medical man and Wilde the Irish medical man-the historian of Irish medical traditions and the biographer of Irish medical men, and William Wilde as an Irish Victorian. A variety of close British Isles parallels can be drawn between Wilde and his cohort in the medical elite of Dublin and their clinical peers in Edinburgh and London both in terms of clinical practice and self-presentation and in terms of the social and political challenges facing their respective ancient regime hegemonies in an age of democratic radicalisation. The shared ideological interests of Wilde and his cohort, however, were also challenged by the socio-political particularities and complexities of Ireland during the first half of the nineteenth century culminating in the catastrophe of the Great Famine. William Wilde saw the practice of scientific medicine as offering a means of deliverance from historical catastrophe for Irish society and invoked a specifically Irish scientific and medical tradition going back to the engagement with the condition of Ireland by enlightened medical men in the seventeenth and eighteenth centuries.

  15. Cardiac arrest related to anaesthesia in Williams-Beuren syndrome.

    PubMed

    Lucena Delgado, J; Sanabria Carretero, P; Durán la Fuente, P; Gónzalez Rocafort, A; Castro Parga, L; Reinoso Barbero, F

    2018-04-01

    Williams-Beuren syndrome is the clinical manifestation of a congenital genetic disorder in the elastin gene, among others. There is a history of cardiac arrest refractory to resuscitation manoeuvres in anaesthesia. The incidence of myocardial ischaemia is high during anaesthetic induction, but there are patients who do not have this condition yet also have had very serious cardiac events, and issues that are still to be resolved. Case descriptions will enable the common pathophysiological factors to be defined, and decrease morbidity and mortality. We report the case of a 3-year-old boy with cardiac arrest at induction, rescued with circulatory assistance with extracorporeal membrane oxygenation and hypothermia induced for cerebral protection. Copyright © 2017 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.

  16. Williams in Node 1 / Unity module

    NASA Image and Video Library

    2007-03-01

    ISS014-E-15830 (4 March 2007) --- Astronaut Sunita L. Williams, Expedition 14 flight engineer, works with a portion of the Treadmill Vibration Isolation System (TVIS) during in-flight maintenance (IFM) in the Unity node of the International Space Station.

  17. 40 CFR Appendix B to Part 425 - Modified Monier-Williams Method

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 40 Protection of Environment 30 2011-07-01 2011-07-01 false Modified Monier-Williams Method B... Part 425—Modified Monier-Williams Method Outline of Method Hydrogen sulfide is liberated from an.... Quality Control 1. Each laboratory that uses this method is required to operate a formal quality control...

  18. 40 CFR Appendix B to Part 425 - Modified Monier-Williams Method

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 40 Protection of Environment 29 2010-07-01 2010-07-01 false Modified Monier-Williams Method B... Part 425—Modified Monier-Williams Method Outline of Method Hydrogen sulfide is liberated from an.... Quality Control 1. Each laboratory that uses this method is required to operate a formal quality control...

  19. 78 FR 11857 - Agency Information Collection Activities; Comment Request; William D. Ford Federal Direct Loan...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-02-20

    ...; Comment Request; William D. Ford Federal Direct Loan Program (DL) Regulations AGENCY: Department of... in response to this notice will be considered public records. Title of Collection: William D. Ford.... Abstract: The William D. Ford Federal Direct Loan Program regulations cover areas of program administration...

  20. 40 CFR Appendix B to Part 425 - Modified Monier-Williams Method

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 40 Protection of Environment 31 2012-07-01 2012-07-01 false Modified Monier-Williams Method B... Appendix B to Part 425—Modified Monier-Williams Method Outline of Method Hydrogen sulfide is liberated from.... Quality Control 1. Each laboratory that uses this method is required to operate a formal quality control...

  1. Solomon Receives 2007 William Bowie Medal

    NASA Astrophysics Data System (ADS)

    Brasseur, Guy; Solomon, Susan

    2008-01-01

    Susan Solomon was awarded the 2007 William Bowie Medal at the AGU Fall Meeting Honors Ceremony, which was held on 12 December 2007 in San Francisco, Calif. The medal is for ``outstanding contributions to fundamental geophysics and for unselfish cooperation in research.''

  2. 75 FR 81640 - President William Jefferson Clinton Birthplace Home National Historic Site

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-12-28

    ... Jefferson Clinton Birthplace Home National Historic Site AGENCY: National Park Service, Interior. ACTION..., Hope, Arkansas 71801, as the ``President William Jefferson Clinton Birthplace Home National Historic... Birthplace Foundation, Inc., fee simple, unencumbered title to the William Jefferson Clinton Birthplace Home...

  3. The Williams' legacy: A critical reappraisal of his nine predictions about the evolution of senescence.

    PubMed

    Gaillard, Jean-Michel; Lemaître, Jean-François

    2017-12-01

    Williams' evolutionary theory of senescence based on antagonistic pleiotropy has become a landmark in evolutionary biology, and more recently in biogerontology and evolutionary medicine. In his original article, Williams launched a set of nine "testable deductions" from his theory. Although some of these predictions have been repeatedly discussed, most have been overlooked and no systematic evaluation of the whole set of Williams' original predictions has been performed. For the sixtieth anniversary of the publication of the Williams' article, we provide an updated evaluation of all these predictions. We present the pros and cons of each prediction based on recent accumulation of both theoretical and empirical studies performed in the laboratory and in the wild. From our viewpoint, six predictions are mostly supported by our current knowledge at least under some conditions (although Williams' theory cannot thoroughly explain why for some of them). Three predictions, all involving the timing of senescence, are not supported. Our critical review of Williams' predictions highlights the importance of William's contribution and clearly demonstrates that, 60 years after its publication, his article does not show any sign of senescence. © 2017 The Author(s). Evolution © 2017 The Society for the Study of Evolution.

  4. Ocular Features in 16 Brazilian Patients with Williams-Beuren Syndrome.

    PubMed

    Viana, Melissa Machado; Frasson, Maria; Galvão, Henrique; Leão, Letícia Lima; Stofanko, Martin; Gonçalves-Dornelas, Higgor; da Silva Cunha, Pricila; Burle de Aguiar, Marcos José

    2015-01-01

    Williams-Beuren Syndrome (WBS) is a multisystem disorder caused by the deletion of contiguous genes on chromosome 7q11.23. Ophthalmologic abnormalities and deficits in visual motor integration are important features of WBS. Here we describe our experience with Brazilian WBS patients and their ophthalmologic features. Sixteen patients with confirmed WBS went through thorough ophthalmologic examination. The most frequent ocular findings in our group of patients were stellate iris pattern (81.2%), hyperopic astigmatism (50%), hyperopia (37.5%), tortuosity of retinal vessel (37.5%) and strabismus (18.7%). This is the second report of ophthalmologic abnormalities in a group of Brazilian individuals with WBS. It is extremely valuable that specific populations are studied so that clinical diagnosis can be refined and management of patients can be driven to the most common presentations of the disease.

  5. Music and Anxiety in Williams Syndrome: A Harmonious or Discordant Relationship?

    ERIC Educational Resources Information Center

    Dykens, Elisabeth M.; Rosner, Beth A.; Ly, Tran; Sagun, Jaclyn

    2005-01-01

    In this two-part study, we assessed musical involvements in two samples of persons with Williams syndrome compared to others with mental retardation and also related musicality to anxiety and fears in Study 2. Relative to others with mental retardation, those with Williams syndrome were more likely to take music lessons, play an instrument, and…

  6. Walter C. Williams (1919-1995)

    NASA Technical Reports Server (NTRS)

    1954-01-01

    Walter C. Williams was Chief of the National Advisory Committee for Aeronautics' and the National Aeronautics and Space Administration's flight research organization on Edwards Air Force Base until his appointment as Associate Director of Project Mercury on September 15, 1959. Walt had started his career with NACA at Langley Memorial Aeronautical Laboratory in 1939 as an engineer in the Flight Division. In 1946 he transferred to the Muroc Army Air Field to be in charge of the small group of technicians and engineers who would be doing the flight research on a joint NACA-Army Air Forces program involving the rocket-powered Bell XS-1. See photo DIRECTORS E-49-0170, which addresses the first eight years of Walt's responsibilities with NACA. Williams' achievements as Chief of the NACA/NASA High-Speed Flight Station for the next five years continued to be significant. NACA pilot Joseph A. Walker made the first of 20 NACA research flights in the Douglas X-3 'Flying Stiletto'--on which inertial coupling was first experience--in 1954. The first NACA flight in an Lockheed F-104A aircraft occurred on August 27, 1956. On October 15, 1958, the first of three North American X-15 rocket research aircraft arrived at NASA High Speed Flight Station as preparations moved ahead for the highly successful NASA-Air Force-Navy-North American program that would last 10 years and investigate hypersonic flight. Walt directed a great variety of other flight research programs, including that on the Boeing B-47; investigations using the Century Series fighters, F-100, F-102, F-104, F-105 and F-107; and the ones involving the X-1 #2, which became the X1-E. During Williams' career, he twice received the NASA Distinguished Service Medal and was nominated both to the Meritorious Rank and Distinguished Rank in the Federal Senior Executive Service. In 1963 he was awarded an honorary doctorate of engineering degree by Louisiana State University. He received several awards from the American Institute

  7. Who Was the Real William Shakespeare?

    ERIC Educational Resources Information Center

    Edwards, Michael Todd

    2009-01-01

    This article highlights a project that encourages students to connect reading and mathematics instruction by using a data analysis approach. Students analyze sonnets from statistical, literary, and historical points of view in an effort to uncover the true identity of William Shakespeare. (Contains 10 figures.)

  8. Mr. William Shakespeare and the Internet.

    ERIC Educational Resources Information Center

    Reeves, Barbara

    2000-01-01

    Describes resources and links on a Web site entitled "Mr. William Shakespeare and the Internet," with suggestions for using them with students. Highlights include: historical context; impact of events/situations on works; motivational/preparatory lessons; reading and understanding Shakespeare; analysis of works; language; assessing other…

  9. MacWilliams Identity for M-Spotty Weight Enumerator

    NASA Astrophysics Data System (ADS)

    Suzuki, Kazuyoshi; Fujiwara, Eiji

    M-spotty byte error control codes are very effective for correcting/detecting errors in semiconductor memory systems that employ recent high-density RAM chips with wide I/O data (e.g., 8, 16, or 32bits). In this case, the width of the I/O data is one byte. A spotty byte error is defined as random t-bit errors within a byte of length b bits, where 1 le t ≤ b. Then, an error is called an m-spotty byte error if at least one spotty byte error is present in a byte. M-spotty byte error control codes are characterized by the m-spotty distance, which includes the Hamming distance as a special case for t =1 or t = b. The MacWilliams identity provides the relationship between the weight distribution of a code and that of its dual code. The present paper presents the MacWilliams identity for the m-spotty weight enumerator of m-spotty byte error control codes. In addition, the present paper clarifies that the indicated identity includes the MacWilliams identity for the Hamming weight enumerator as a special case.

  10. Sir William Hingston

    PubMed Central

    Cohen, Jack

    1996-01-01

    Sir William Hingston was one of Canada’s most illustrious surgeons in the second half of the 19th century. Not only was he a very innovative surgeon but he was an excellent teacher and wrote many medical articles during a career that spanned over 50 years. Active as he was medically, he found time to serve a term as mayor of Montreal and was on the board of directors of various banks and companies. As recognition of his many talents, he was knighted by Queen Victoria in 1895. He died in 1907 at the age of 78 years. PMID:8857994

  11. Sir William Osler revisited.

    PubMed Central

    Duffy, T. P.

    1980-01-01

    The basis of Sir William Osler's fame is elusive to almost all and the appropriateness of such recognition is questioned by many. His many contributions as a practitioner, teacher, writer, and scientist in medicine do not adequately explain his prominence 60 years following his death. It was his participation in the covenant of medicine and the special components of that relationship that may account for his hold on his followers today. Images FIG. 1 FIG. 2 PMID:6996344

  12. [Vascular malformations in the Williams-Beuren syndrome: report of three new cases].

    PubMed

    Sator, Hicham; Rhouni, Fatima Ezzahra; Benjouad, Ibitihale; Rhouni, Fatima Ezzahra; Benjouad, Ibitihale; Dafiri, Rachida; Chat, Latifa

    2016-01-01

    The Williams-Beuren syndrome is a rare genetic disease. It combines classically specific facial dysmorphism, cardiovascular malformations and specific neuropsychological profile. We report three cases of Williams-Beuren syndrome in children with particular emphasis on vascular abnormalities observed on CT angiography and MR angiography.

  13. 75 FR 22436 - Michael Williams-Control Exemption-St. Maries River Railroad, Inc.

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-04-28

    ... DEPARTMENT OF TRANSPORTATION Surface Transportation Board [Docket No. FD 35365] Michael Williams-Control Exemption-St. Maries River Railroad, Inc. Michael Williams (applicant),\\1\\ a noncarrier, has filed a verified notice of exemption to acquire control of St. Maries River Railroad, Inc. (STMA), a Class...

  14. William Wolfgang Brickman, 1913-86.

    ERIC Educational Resources Information Center

    Parker, Franklin

    William Wolfgang Brickman, founding member and President, 1956-59, of the Comparative and International Education Society, died June 22, 1986, in a Philadelphia hospital leukemia unit. Born June 30, 1913, in New York City, he attended city schools and earned B.A. and M.S. degrees at City College, a New York University Ph.D. and an honorary M.A.…

  15. Stranger Danger Awareness in Williams Syndrome

    ERIC Educational Resources Information Center

    Riby, D. M.; Kirk, H.; Hanley, M.; Riby, L. M.

    2014-01-01

    Background: The developmental disorder Williams syndrome (WS) is characterised by a distinctive cognitive profile and an intriguing social phenotype. Individuals with the disorder are often highly social engaging with familiar and unfamiliar people and once in an interaction they often show subtle abnormalities of social behaviour. Atypically…

  16. 250 Years of Physics at the College of William and Mary: 1760-2010

    NASA Astrophysics Data System (ADS)

    von Baeyer, Hans

    2010-02-01

    The recorded history of physics at William and Mary begins when Thomas Jefferson, the College's most distinguished alumnus, meets his mentor, Dr. William Small of Scotland, who opens his eyes to the wonders of natural philosophy. After the vicissitudes of the Revolution and the Civil War, physics enjoys a revival in the twentieth century, culminating in the creation of a Ph.D. program in the 1960s and the building of the William Small Physical Laboratory in Williamsburg. In the 1980s the modern era is launched by the establishment of the US Department of Energy's Jefferson Lab for nuclear physics in nearby Newport News. Today both Small Hall and Jefferson Lab are in the process of renovation. The legacies of Small and Jefferson for physics at William and Mary are secure! )

  17. 33 CFR 164.43 - Automatic Identification System Shipborne Equipment-Prince William Sound.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 33 Navigation and Navigable Waters 2 2012-07-01 2012-07-01 false Automatic Identification System Shipborne Equipment-Prince William Sound. 164.43 Section 164.43 Navigation and Navigable Waters COAST GUARD... Automatic Identification System Shipborne Equipment—Prince William Sound. (a) Until December 31, 2004, each...

  18. 33 CFR 164.43 - Automatic Identification System Shipborne Equipment-Prince William Sound.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 33 Navigation and Navigable Waters 2 2013-07-01 2013-07-01 false Automatic Identification System Shipborne Equipment-Prince William Sound. 164.43 Section 164.43 Navigation and Navigable Waters COAST GUARD... Automatic Identification System Shipborne Equipment—Prince William Sound. (a) Until December 31, 2004, each...

  19. 33 CFR 164.43 - Automatic Identification System Shipborne Equipment-Prince William Sound.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 33 Navigation and Navigable Waters 2 2014-07-01 2014-07-01 false Automatic Identification System Shipborne Equipment-Prince William Sound. 164.43 Section 164.43 Navigation and Navigable Waters COAST GUARD... Automatic Identification System Shipborne Equipment—Prince William Sound. (a) Until December 31, 2004, each...

  20. GTF2IRD2 is located in the Williams-Beuren syndrome critical region 7q11.23 and encodes a protein with two TFII-I-like helix-loop-helix repeats.

    PubMed

    Makeyev, Aleksandr V; Erdenechimeg, Lkhamsuren; Mungunsukh, Ognoon; Roth, Jutta J; Enkhmandakh, Badam; Ruddle, Frank H; Bayarsaihan, Dashzeveg

    2004-07-27

    Williams-Beuren syndrome (also known as Williams syndrome) is caused by a deletion of a 1.55- to 1.84-megabase region from chromosome band 7q11.23. GTF2IRD1 and GTF2I, located within this critical region, encode proteins of the TFII-I family with multiple helix-loop-helix domains known as I repeats. In the present work, we characterize a third member, GTF2IRD2, which has sequence and structural similarity to the GTF2I and GTF2IRD1 paralogs. The ORF encodes a protein with several features characteristic of regulatory factors, including two I repeats, two leucine zippers, and a single Cys-2/His-2 zinc finger. The genomic organization of human, baboon, rat, and mouse genes is well conserved. Our exon-by-exon comparison has revealed that GTF2IRD2 is more closely related to GTF2I than to GTF2IRD1 and apparently is derived from the GTF2I sequence. The comparison of GTF2I and GTF2IRD2 genes revealed two distinct regions of homology, indicating that the helix-loop-helix domain structure of the GTF2IRD2 gene has been generated by two independent genomic duplications. We speculate that GTF2I is derived from GTF2IRD1 as a result of local duplication and the further evolution of its structure was associated with its functional specialization. Comparison of genomic sequences surrounding GTF2IRD2 genes in mice and humans allows refinement of the centromeric breakpoint position of the primate-specific inversion within the Williams-Beuren syndrome critical region.

  1. N-acetylcysteine for neuropsychiatric symptoms in a woman with Williams syndrome.

    PubMed

    Pineiro, Mildred Lopez; Roberts, Antoinette M; Waxler, Jessica L; Mullett, Jennifer E; Pober, Barbara R; McDougle, Christopher J

    2014-11-01

    Williams syndrome is a relatively rare genetic disorder caused by the hemizygous microdeletion of a region in chromosome 7q11.23. Individuals with Williams syndrome typically present with a highly social, overfriendly, and empathic personality. Comorbid medical and neuropsychiatric disorders are common. Reports of effective pharmacological treatment of associated neuropsychiatric disorders are limited. The authors describe the successful treatment of interfering anger, aggression, and hair-pulling with N-acetylcysteine in a 19-year-old woman with Williams syndrome. The neuropsychiatric symptoms emerged 1 week following an upper gastrointestinal endoscopy, for which fentanyl, midazolam, and propofol were used as anesthetics. The patient's treatment course and hypothesized mechanisms underlying the clinical presentation and symptom resolution are described. © The Author(s) 2014.

  2. Williams receives a haircut from Yurchikhin in the SM during Expedition 15

    NASA Image and Video Library

    2007-06-03

    ISS015-E-10596 (3 June 2007) --- Cosmonaut Fyodor N. Yurchikhin, Expedition 15 commander representing Russia's Federal Space Agency, cuts astronaut Sunita L. Williams' hair in the Zvezda Service Module of the International Space Station. Williams, flight engineer, holds a vacuum device fashioned to garner freshly cut hair.

  3. Williams receives a haircut from Yurchikhin in the SM during Expedition 15

    NASA Image and Video Library

    2007-06-03

    ISS015-E-10595 (3 June 2007) --- Cosmonaut Fyodor N. Yurchikhin, Expedition 15 commander representing Russia's Federal Space Agency, cuts astronaut Sunita L. Williams' hair in the Zvezda Service Module of the International Space Station. Williams, flight engineer, holds a vacuum device fashioned to garner freshly cut hair.

  4. William E. Wilson and his contemporaries

    NASA Astrophysics Data System (ADS)

    Elliott, I.

    Although he never attended school or university, William E. Wilson FRS, of Daramona, County Westmeath, made pioneering contributions to solar physics, celestial photography and stellar photometry. His well-equipped observatory attracted collaborators who included George Francis FitzGerald of Trinity College Dublin and Arthur Rambaut of Dunsink Observatory.

  5. Fading-Figure Tracing in Williams Syndrome

    ERIC Educational Resources Information Center

    Nagai, Chiyoko; Inui, Toshio; Iwata, Makoto

    2011-01-01

    Williams syndrome (WS) is a neurodevelopmental disorder characterized by severe impairment of visuospatial abilities. Figure-drawing abilities, which are thought to reflect visuospatial abilities, have yet to be fully investigated in WS. The purpose of the present study was to clarify whether drawing abilities differ between WS individuals and…

  6. Rare genomic rearrangement in a boy with Williams-Beuren syndrome associated to XYY syndrome and intriguing behavior.

    PubMed

    Dutra, Roberta L; Piazzon, Flavia B; Zanardo, Évelin A; Costa, Thais Virginia Moura Machado; Montenegro, Marília M; Novo-Filho, Gil M; Dias, Alexandre T; Nascimento, Amom M; Kim, Chong Ae; Kulikowski, Leslie D

    2015-12-01

    Williams-Beuren syndrome (WBS) is caused by a hemizygous contiguous gene microdeletion of 1.55-1.84 Mb at 7q11.23 region. Approximately, 28 genes have been shown to contribute to classical phenotype of SWB with presence of dysmorphic facial features, supravalvular aortic stenosis (SVAS), intellectual disability, and overfriendliness. With the use of Microarray-based comparative genomic hybridization and other molecular cytogenetic techniques, is possible define with more accuracy partial or atypical deletion and refine the genotype-phenotype correlation. Here, we report on a rare genomic structural rearrangement in a boy with atypical deletion in 7q11.23 and XYY syndrome with characteristic clinical signs, but not sufficient for the diagnosis of WBS. Cytogenetic analysis of G-banding showed a karyotype 47,XYY. Analysis of DNA with the technique of MLPA (Multiplex Ligation-dependent Probe Amplification) using kits a combination of kits (P064, P036, P070, and P029) identified an atypical deletion on 7q11.23. In addition, high resolution SNP Oligonucleotide Microarray Analysis (SNP-array) confirmed the alterations found by MLPA and revealed others pathogenic CNVs, in the chromosomes 7 and X. The present report demonstrates an association not yet described in literature, between Williams-Beuren syndrome and 47,XYY. The identification of atypical deletion in 7q11.23 concomitant to additional pathogenic CNVs in others genomic regions allows a better comprehension of clinical consequences of atypical genomic rearrangements. © 2015 Wiley Periodicals, Inc.

  7. Seasonal distribution of Dall's porpoise in Prince William Sound, Alaska

    NASA Astrophysics Data System (ADS)

    Moran, John R.; O'Dell, Matthew B.; Arimitsu, Mayumi L.; Straley, Janice M.; Dickson, Danielle M. S.

    2018-01-01

    Dall's porpoise, Phocoenoides dalli, are a conspicuous predator in the Prince William Sound ecosystem, yet there has been little effort directed towards monitoring this species since the 1980s, prior to the Exxon Valdez oil spill. We used vessel-based surveys to examine the seasonal distribution of Dall's porpoise in the waters of Prince William Sound during eight years from 2007 to 2015. Over the course of 168 days and 15,653 km of survey effort, 921 Dall's porpoise were encountered in 210 groups. We estimate an encounter rate of 0.061 porpoise/km traveled or 1 porpoise encountered for every 16.5 km traveled. Dall's porpoise were found throughout the year in Prince William Sound, and used a wide range of habitats, including those not considered typical of the species, such as bays, shallow water, and nearshore waters. Dall's porpoise seasonally shifted their center of distribution from the western passages in fall to the bays of the eastern Sound in winter and spring. Dall's porpoises were widely dispersed throughout the Sound in summer. We identified potential Dall's porpoise habitat (depth, slope, and distance from shore) within Prince William Sound using generalized additive models (GAM). Dall's porpoise were found in deeper water during summer and in shallowest water during spring. We propose that their use of novel habitats is a function of reduced predation risk associated with the decline of their main predator, killer whales (Orcinus orca), following the Exxon Valdez oil spill, and the presence of overwintering and spawning Pacific herring (Clupea pallasii). While the size of the Dall's porpoise population within Prince William Sound remains unknown, our encounter rates were lower than those reported in the 1970s. Their high metabolic rate and ubiquitous presence makes them one of the more important, yet understudied, forage fish predators in the region.

  8. Williams-Beuren syndrome associated with single kidney and nephrocalcinosis: a case report.

    PubMed

    Abidi, Kamel; Jellouli, Manel; Ben Rabeh, Rania; Hammi, Yousra; Gargah, Tahar

    2015-01-01

    Williams-Beuren syndrome is a rare neurodevelopmental disorder, characterized by congenital heart defects, abnormal facial features, mental retardation with specific cognitive and behavioral profile, growth hormone deficiency, renal and skeletal anomalies, inguinal hernia, infantile hypercalcaemia. We report a case with Williams-Beuren syndrome associated with a single kidney and nephrocalcinosis complicated by hypercalcaemia. A male infant, aged 20 months presented growth retardation associated with a psychomotor impairment, dysmorphic features and nephrocalcinosis. He had also hypercalciuria and hypercalcemia. Echocardiography was normal. DMSA renal scintigraphy showed a single functioning kidney. The FISH generated one ELN signal in 20 metaphases read and found the presence of ELN deletion, with compatible Williams-Beuren syndrome.

  9. ASK Talks with William Readdy

    NASA Technical Reports Server (NTRS)

    2004-01-01

    An interview with William Readdy is presented.Rsaddy graduated From the United States Naval Academy in 1974. After eleven years service as a naval aviator and test pilot, he joined NASA in 1986 as a research pilot. His technical assignments to date have included Training and Safety Officer, Orbiter project staff; NASA Director of Operations in Star City, Russia; and Space Shuttle Program Development Manager.

  10. MRI Amygdala Volume in Williams Syndrome

    ERIC Educational Resources Information Center

    Capitao, Liliana; Sampaio, Adriana; Sampaio, Cassandra; Vasconcelos, Cristiana; Fernandez, Montse; Garayzabal, Elena; Shenton, Martha E.; Goncalves, Oscar F.

    2011-01-01

    One of the most intriguing characteristics of Williams Syndrome individuals is their hypersociability. The amygdala has been consistently implicated in the etiology of this social profile, particularly given its role in emotional and social behavior. This study examined amygdala volume and symmetry in WS individuals and in age and sex matched…

  11. Attention to Faces in Williams Syndrome

    ERIC Educational Resources Information Center

    Riby, Deborah M.; Jones, Nicola; Brown, Philippa H.; Robinson, Lucy J.; Langton, Stephen R. H.; Bruce, Vicki; Riby, Leigh M.

    2011-01-01

    Williams syndrome (WS) is associated with distinct social behaviours. One component of the WS social phenotype is atypically prolonged face fixation. This behaviour co-exists with attention difficulties. Attention is multi-faceted and may impact on gaze behaviour in several ways. Four experiments assessed (i) attention capture by faces, (ii)…

  12. A Conversation with William Van Til.

    ERIC Educational Resources Information Center

    Beineke, John A.

    1989-01-01

    Reports an interview with William Van Til, Coffman Distinguished Professor Emeritus of Education at Indiana State University (Terre Haute). Van Til reviews his career as a social studies teacher and his writings on curriculum and social foundations. Presents his opinions on progressive education, famous educators, educational history and reform,…

  13. William M. Harnett: "After the Hunt."

    ERIC Educational Resources Information Center

    Davidson, Edie

    1987-01-01

    Using William M. Harnett's 1884 oil painting "After the Hunt," this lesson introduces students in grades seven through nine to the style of realism popular in the late nineteenth century. The lesson is also designed to show how art reflects the values of the society in which it was created. (JDH)

  14. Brief Report: Repetitive Behaviour Profiles in Williams syndrome: Cross Syndrome Comparisons with Prader-Willi and Down syndromes.

    PubMed

    Royston, R; Oliver, C; Moss, J; Adams, D; Berg, K; Burbidge, C; Howlin, P; Nelson, L; Stinton, C; Waite, J

    2018-01-01

    This study describes the profile of repetitive behaviour in individuals with Williams syndrome, utilising cross-syndrome comparisons with people with Prader-Willi and Down syndromes. The Repetitive Behaviour Questionnaire was administered to caregivers of adults with Williams (n = 96), Prader-Willi (n = 103) and Down (n = 78) syndromes. There were few group differences, although participants with Williams syndrome were more likely to show body stereotypies. Individuals with Williams syndrome also showed more hoarding and less tidying behaviours than those with Down syndrome. IQ and adaptive ability were negatively associated with repetitive questioning in people with Williams syndrome. The profile of repetitive behaviour amongst individuals with Williams syndrome was similar to the comparison syndromes. The cognitive mechanisms underlying these behaviours in genetic syndromes warrant further investigation.

  15. Recent KBO (Pluto/Charon and beyond, including Quaoar) Occultation Observations by the Williams College Team as part of the Williams-MIT Collaboration

    NASA Astrophysics Data System (ADS)

    Pasachoff, Jay M.; Babcock, B. A.; Davis, A. B.; Pandey, S.; Lu, M.; Rogosinski, Z.; Person, M. J.; Bosh, A. S.; Zangari, A. M.; Zuluaga, C. A.; Gulbis, A. S.; Naranjo, O.; Navas, G.; Zerpa, L.; Villarreal, J.; Rojo, P.; Förster, F.; Servajean, E.

    2013-10-01

    The Williams College-MIT collaboration has observed numerous occultations of stars by Pluto/Charon and other Kuiper-belt objects (www.stellaroccultations.info), since its establishment three decades ago with an attempted discovery of Neptune's rings in 1983. In this paper, we describe several recent occultation observations, both successful and (for reasons of path uncertainties and/or weather) unsuccessful. Light curves made or arranged by Williams College faculty and students were used together with light curves by MIT colleagues and others to study Pluto's atmosphere and Charon's size, to discover one of the highest-known solar-system albedos (KBO 55636), and to attempt to study 1000-km-diameter Quaoar. Observations discussed include light curves for KBO 55636 on 9 October 2009 from Hawaii; Pluto on 3/4 July 2010 from Chile, 22 May 2011 from Williamstown, Massachusetts, 23 June 2011 from Hawaii (in support of SOFIA observations of Pluto's atmosphere, discussed in an article in press in AJ and of the pair of Pluto/Charon occultations of the same star), and 4 May 2013 (Bosh et al., this conference) and 15 July 2013 from Williamstown; Charon on 15 June 2013 from Williamstown; Quaoar from a picket fence ranging from Chile through Venezuela (with a detection there) to Massachusetts on July 8/9 and in South Africa on 12 July 2013. This work was supported in part by NASA Planetary Astronomy grants NNX08AO50G and NNH11ZDA001N to Williams College, NNX10AB27G to MIT, and USRA grant #8500-98-003 to Lowell Observatory. We thank Steven P. Souza at Williams; Steven Levine at Lowell Obs.; Jennifer G. Winters (GSU) in Chile; Richard Rojas/Jorge Moreno in Venezuela; Scott Sheppard; Federica Bianco; David Osip; and others. ZR (Vassar '14) was a Keck Northeast Astronomy Consortium Summer Fellow at Williams College, supported by an NSF/REU grant to the Keck Northeast Astronomy Consortium. ES: partial support from Programa Nacional de Becas de Postgrado (CONICYT Grant 21110496). FF

  16. The Pennsylvania Anatomy Act of 1883: Weighing the Roles of Professor William Smith Forbes and Senator William James McKnight.

    PubMed

    Wright, James R

    2016-10-01

    Effective Anatomical Acts transformed medical education and curtailed grave-robbing. William S. Forbes, Demonstrator of Anatomy at Jefferson Medical College in Philadelphia, authored the Pennsylvania Anatomy Act of 1867, but it was ineffective. In December of 1882, Forbes and accomplices were charged with grave-robbing. Forbes was acquitted in early 1883, but his accomplices were all convicted; nevertheless, these events precipitated a strengthened Anatomy Act in 1883. Forbes was crowned the Father of the Pennsylvania Anatomy Act and was revered by the Philadelphia medical community for his personal sacrifices for medical education; they even paid his legal fees. Over the remainder of his life, Forbes received many honors. However, there was a second major player, rural doctor William J. McKnight, a convicted grave-robber and State Senator. The evidence shows that Forbes precipitated the crisis, which was a racial powder keg, and then primarily focused on his trial, while McKnight, creatively working behind the scenes in collaboration with Jefferson, Anatomy Professor William H. Pancoast, used the crisis to draft and pass transformative legislation enabling anatomical dissection at Pennsylvania medical schools. While not minimizing Forbes suffering throughout these events, McKnight should be appropriately recognized for his initiative and contributions, which far exceeded those of Forbes. © The Author 2016. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  17. Outcome in Adult Life for People with Williams Syndrome Results from a Survey of 239 Families

    ERIC Educational Resources Information Center

    Howlin, P.; Udwin, O.

    2006-01-01

    BACKGROUND: Although there has been considerable research into the genotype and phenotype of Williams syndrome, there have been relatively few studies of long-term prognosis. As a preliminary to a more detailed investigation of adults with Williams syndrome, a parental questionnaire was distributed to members of the UK Williams Syndrome…

  18. Holocene deposition and megathrust splay fault geometries within Prince William Sound, Alaska

    NASA Astrophysics Data System (ADS)

    Finn, S.; Liberty, L. M.; Haeussler, P. J.; Pratt, T. L.

    2011-12-01

    New high resolution sparker seismic reflection data, in conjunction with reprocessed legacy seismic data, provide the basis for a new fault, fold, and Holocene sediment thickness database for Prince William Sound, Alaska. Additionally, legacy airgun seismic data in Prince William Sound and the Gulf of Alaska tie features on these new sparker data to deeper portions of megathrust splay faults. We correlate regionally extensive bathymetric lineaments within Prince William Sound to megathrust splay faults, such as the ones that ruptured in the 1964 M9.2 earthquake. Lastly, we estimate Holocene sediment thickness within Prince William Sound to better constrain the Holocene fault history throughout the region. We identify three seismic facies related to Holocene, Quaternary, and Tertiary strata that are crosscut by numerous high angle normal faults in the hanging wall of the megathrust splay faults. The crustal-scale seismic reflection profiles show splay faults emerging from 20 km depth between the Yakutat block and North American crust and surfacing as the Hanning Bay and Patton Bay faults. A change in exhumation rates, slip rates, and fault orientation appears near Hinchinbrook that we attribute to differences in subducted slab geometry. Based on our slip rate analysis, we calculate average Holocene displacements of 20 m and 100 m in eastern and western Prince William Sound, respectively. Landward of two splay faults exposed on Montague Island, we observe subsidence, faulting, and landslides that record deformation associated with the 1964 and older megathrust earthquakes.

  19. Attentional Disengagement in Adults with Williams Syndrome

    ERIC Educational Resources Information Center

    Lense, Miriam D.; Key, Alexandra P.; Dykens, Elisabeth M.

    2011-01-01

    Williams syndrome (WS) is a neurodevelopmental disorder characterized by a distinctive behavioral and cognitive profile, including widespread problems with attention. However, the specific nature of their attentional difficulties, such as inappropriate attentional allocation and/or poor attentional disengagement abilities, has yet to be…

  20. Sleep-EEG in dizygotic twins discordant for Williams syndrome.

    PubMed

    Bódizs, Róbert; Gombos, Ferenc; Szocs, Katalin; Réthelyi, János M; Gerván, Patrícia; Kovács, Ilona

    2014-01-30

    Reports on twin pairs concordant and discordant for Williams syndrome were published before, but no study unravelled sleep physiology in these cases yet. We aim to fill this gap by analyzing sleep records of a twin pair discordant for Williams syndrome extending our focus on presleep wakefulness and sleep spindling. We performed multiplex ligation-dependent probe amplification of the 7q11.23 region of a 17 years old dizygotic opposite-sex twin pair discordant for Williams syndrome. Polysomnography of laboratory sleep at this age was analyzed and followed-up after 1.5 years by ambulatory polysomnography. Sleep stages scoring, EEG power spectra and sleep spindle analyses were carried out. The twin brother showed reduced levels of amplification for all of the probes in the 7q11.23 region indicating a typical deletion spanning at least 1.038 Mb between FKBP6 and CLIP2. The results of the twin sister showed normal copy numbers in the investigated region. Lower sleep times and efficiencies, as well as higher slow wave sleep percents of the twin brother were evident during both recordings. Roughly equal NREM, Stage 2 and REM sleep percents were found. EEG analyses revealed state and derivation-independent decreases in alpha power, lack of an alpha spectral peak in presleep wakefulness, as well as higher NREM sleep sigma peak frequency in the twin brother. Faster sleep spindles with lower amplitude and shorter duration characterized the records of the twin brother. Spectra show a striking reliability and correspondence between the two situations (laboratory vs. home records). Alterations in sleep and specific neural oscillations including the alpha/sigma waves are inherent aspects of Williams syndrome.

  1. Comparison of the CEAS and Williams-type barley yield models for North Dakota and Minnesota

    NASA Technical Reports Server (NTRS)

    Leduc, S. (Principal Investigator)

    1982-01-01

    The CEAS and Williams type models were compared based on specified selection criteria which includes a ten year bootstrap test (1970-1979). Based on this, the models were quite comparable; however, the CEAS model was slightly better overall. The Williams type model seemed better for the 1974 estimates. Because that year spring wheat yield was particularly low, the Williams type model should not be excluded from further consideration.

  2. Auditory Attraction: Activation of Visual Cortex by Music and Sound in Williams Syndrome

    ERIC Educational Resources Information Center

    Thornton-Wells, Tricia A.; Cannistraci, Christopher J.; Anderson, Adam W.; Kim, Chai-Youn; Eapen, Mariam; Gore, John C.; Blake, Randolph; Dykens, Elisabeth M.

    2010-01-01

    Williams syndrome is a genetic neurodevelopmental disorder with a distinctive phenotype, including cognitive-linguistic features, nonsocial anxiety, and a strong attraction to music. We performed functional MRI studies examining brain responses to musical and other types of auditory stimuli in young adults with Williams syndrome and typically…

  3. WILLIAM GOLDING'S NOVEL--THE BACKWARD LOOK.

    ERIC Educational Resources Information Center

    PECK, CAROL FAULKNER

    THE "SURPRISE ENDINGS" IN EACH OF WILLIAM GOLDING'S FIRST FOUR NOVELS OCCUR WHEN THE POINT OF VIEW SHIFTS FROM THE LIMITED WORLD OF THE NOVEL TO THE UNLIMITED WORLD OF REALITY. THE BOYS' RESCUE BY THE UNCOMPREHENDING OFFICER IN "LORD OF THE FLIES," REFOCUSES AND REINFORCES ALL THAT PRECEDES IT, AND THE FABLE, SUPERIMPOSED UPON REAL LIFE, BECOMES…

  4. Williams with VO2max

    NASA Image and Video Library

    2012-08-08

    ISS032-E-016876 (8 Aug. 2012) --- NASA astronaut Sunita Williams, Expedition 32 flight engineer, performs a VO2max experiment while using the Cycle Ergometer with Vibration Isolation System (CEVIS) in the Destiny laboratory of the International Space Station. VO2max uses the Portable Pulmonary Function System (PPFS), CEVIS, Pulmonary Function System (PFS) gas cylinders and mixing bag system, plus multiple other pieces of hardware to measure oxygen uptake and cardiac output.

  5. A TRIBUTE TO DR. WILLIAM PENN WATKINSON

    EPA Science Inventory

    Dr. William Penn Watkinson (known to colleagues as "Penn") of EPA¿s health research lab (National Health and Environmental Research Laboratory) of Research Triangle Park, North Carolina, died Wednesday, December 13 after a battle with lung cancer. He was a member of the Pulmonar...

  6. SHUTTLE - ASTRONAUT-CANDIDATE (ASCAN) WILLIAMS, DONALD (DON)

    NASA Image and Video Library

    1979-04-03

    S79-30367/69: Exterior views of 747 Shuttle Aircraft. S79-30360/66: ASCAN Don Williams during H Reflex Studies and Hamilton-Standard Tool Belt and Suit Cooling System Tests. 1. AIRCRAFT - 747 2. SHUTTLE - TESTS. S79-30360 thru S79-30369

  7. Parent and teacher perspectives about problem behavior in children with Williams syndrome.

    PubMed

    Klein-Tasman, Bonita P; Lira, Ernesto N; Li-Barber, Kirsten T; Gallo, Frank J; Brei, Natalie G

    2015-01-01

    Problem behavior of 52 children with Williams syndrome ages 6 to 17 years old was examined based on both parent and teacher report. Generally good inter-rater agreement was found. Common areas of problem behavior based both on parent and teacher report included attention problems, anxiety difficulties, repetitive behaviors (e.g., obsessions, compulsions, picking nose or skin), and social problems, reflecting a robust behavioral phenotype in Williams syndrome present across contexts. Some rater differences were observed; most notably, parents reported more attention and mood difficulties than did teachers, while teachers reported more oppositionality and aggression than did parents. Relations to intellectual functioning, age, and gender were examined. The implications of the findings for understanding the behavioral phenotype associated with Williams syndrome are discussed.

  8. Expedition 21 astronauts Jeff Williams and Nicole Stott

    NASA Image and Video Library

    2009-06-24

    Date: 6-24-09 Location: Bldg 9NW - Node 1 Training Area Subject: Expedition 21 astronauts Jeff Williams and Nicole Scott during new Vestibule Procedures training with instructor Michael Steele. Photographer: Lauren Harnett

  9. 78 FR 71022 - Culturally Significant Objects Imported for Exhibition Determinations: “Christopher Williams: The...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-11-27

    ... DEPARTMENT OF STATE [Public Notice 8537] Culturally Significant Objects Imported for Exhibition Determinations: ``Christopher Williams: The Production Line of Happiness'' SUMMARY: Notice is hereby given of the... exhibition ``Christopher Williams: The Product Line of Happiness,'' imported from abroad for temporary...

  10. Robert Owen, William Maclure and New Harmony.

    ERIC Educational Resources Information Center

    Mclaren, David J.

    1996-01-01

    Discusses the utopian philosophy and related educational practices of the early 19th-century communitarian settlement in New Harmony, Indiana. The settlement failed after several years, due in no small part to the falling out between its two main supporters, Robert Owen and William Maclure. (MJP)

  11. Tyurin and Williams in Zvezda Service module

    NASA Image and Video Library

    2007-04-21

    ISS014-E-19924 (21 April 2007) --- Cosmonaut Mikhail Tyurin (left), Expedition 14 flight engineer representing Russia's Federal Space Agency, and astronaut Sunita L. Williams, Expedition 15 flight engineer, drink beverages as they pose for a photo in the Zvezda Service Module of the International Space Station.

  12. Colour discrimination and categorisation in Williams syndrome.

    PubMed

    Farran, Emily K; Cranwell, Matthew B; Alvarez, James; Franklin, Anna

    2013-10-01

    Individuals with Williams syndrome (WS) present with impaired functioning of the dorsal visual stream relative to the ventral visual stream. As such, little attention has been given to ventral stream functions in WS. We investigated colour processing, a predominantly ventral stream function, for the first time in nineteen individuals with Williams syndrome. Colour discrimination was assessed using the Farnsworth-Munsell 100 hue test. Colour categorisation was assessed using a match-to-sample test and a colour naming task. A visual search task was also included as a measure of sensitivity to the size of perceptual colour difference. Results showed that individuals with WS have reduced colour discrimination relative to typically developing participants matched for chronological age; performance was commensurate with a typically developing group matched for non-verbal ability. In contrast, categorisation was typical in WS, although there was some evidence that sensitivity to the size of perceptual colour differences was reduced in this group. Copyright © 2013 Elsevier Ltd. All rights reserved.

  13. Genetic Mapping of Brain Plasticity Across Development in Williams Syndrome: ERP Markers of Face and Language Processing

    PubMed Central

    Mills, D. L.; Dai, L.; Fishman, I.; Yam, A.; Appelbaum, L. G.; Galaburda, A.; Bellugi, U.; Korenberg, J. R.

    2014-01-01

    In Williams Syndrome (WS), a known genetic deletion results in atypical brain function with strengths in face and language processing. We examined how genetic influences on brain activity change with development. In three studies, ERPs from large samples of children, adolescents, and adults with the full genetic deletion for WS were compared to typically developing controls, and two adults with partial deletions for WS. Studies 1 and 2 identified ERP markers of brain plasticity in WS across development. Study 3 suggested that in adults with partial deletions for WS, specific genes may be differentially implicated in face and language processing. PMID:24219698

  14. Williams with TRAC experiment in Destiny

    NASA Image and Video Library

    2007-03-08

    ISS014-E-16215 (8 March 2007) --- Astronaut Sunita L. Williams, Expedition 14 flight engineer, works with the Test of Reaction and Adaptation Capabilities (TRAC) experiment in the Destiny laboratory of the International Space Station. The TRAC investigation will test the theory of brain adaptation during space flight by testing hand-eye coordination before, during and after the space flight.

  15. Williams with TRAC experiment in Destiny

    NASA Image and Video Library

    2007-03-08

    ISS014-E-16210 (8 March 2007) --- Astronaut Sunita L. Williams, Expedition 14 flight engineer, works with the Test of Reaction and Adaptation Capabilities (TRAC) experiment in the Destiny laboratory of the International Space Station. The TRAC investigation will test the theory of brain adaptation during space flight by testing hand-eye coordination before, during and after the space flight.

  16. Williams with TRAC experiment in Destiny

    NASA Image and Video Library

    2007-03-08

    ISS014-E-16214 (8 March 2007) --- Astronaut Sunita L. Williams, Expedition 14 flight engineer, works with the Test of Reaction and Adaptation Capabilities (TRAC) experiment in the Destiny laboratory of the International Space Station. The TRAC investigation will test the theory of brain adaptation during space flight by testing hand-eye coordination before, during and after the space flight.

  17. The Neurophysiology of Autonomic Dysfunction in SCI: Plasticity in the Input and Output Neurons

    DTIC Science & Technology

    2014-04-01

    multi-segmental spinal pain reflex Location: Halls B-H Presentation Time: Tuesday , Nov 16, 2010, 8:00 AM - 9:00 AM Authors: *K. E. TANSEY1, M...neurological scoring methods. Cognitive functions were tested using Fear Conditioning tests at 8–10 days post- injury and Morris Water Maze tests 11–15

  18. Attribution of Negative Intention in Williams Syndrome

    ERIC Educational Resources Information Center

    Godbee, Kali; Porter, Melanie A.

    2013-01-01

    People with Williams syndrome (WS) are said to have sociable and extremely trusting personalities, approaching strangers without hesitation. This study investigated whether people with WS are less likely than controls to attribute negative intent to others when interpreting a series of ambiguous pictures. This may, at least partially, explain…

  19. Executive Function in Williams and Down Syndromes

    ERIC Educational Resources Information Center

    Carney, Daniel P. J.; Brown, Janice H.; Henry, Lucy A.

    2013-01-01

    Williams (WS) and Down (DS) syndromes are characterised by roughly opposing ability profiles. Relative verbal strengths and visuospatial difficulties have been reported in those with WS, while expressive language difficulties have been observed in individuals with DS. Few investigations into the executive function (EF) skills of these groups have…

  20. Analysis of Speech Fluency in Williams Syndrome

    ERIC Educational Resources Information Center

    Rossi, Natalia Freitas; Sampaio, Adriana; Goncalves, Oscar F.; Giacheti, Celia Maria

    2011-01-01

    Williams syndrome (WS) is a neurodevelopmental genetic disorder, often referred as being characterized by dissociation between verbal and non-verbal abilities, although the number of studies disputing this proposal is emerging. Indeed, although they have been traditionally reported as displaying increased speech fluency, this topic has not been…

  1. Pittu Laungani in Conversation with William West

    ERIC Educational Resources Information Center

    Chantler, Khatidja

    2006-01-01

    In this response to Pittu Laungani's conversation with William West, published in the August, 2004, issue of "British Journal of Guidance and Counselling," the author questions why person-centred counselling has been singled out for particular attention, argues that the dichotomy between client-centred and culture-centred counselling is…

  2. Understanding Williams Syndrome: Behavioral Patterns and Interventions.

    ERIC Educational Resources Information Center

    Semel, Eleanor; Rosner, Sue R.

    This guide to Williams syndrome (WS), a congenital disorder characterized by developmental/cognitive limitations but relatively high verbal and social skills, explains the strengths, difficulties and variations found among individuals with the condition and offers guidelines for intervention in the unusual properties of the WS behavioral profile.…

  3. A novel dNTP-limited PCR and HRM assay to detect Williams-Beuren syndrome.

    PubMed

    Zhang, Lichen; Zhang, Xiaoqing; You, Guoling; Yu, Yongguo; Fu, Qihua

    2018-06-01

    Williams-Beuren syndrome (WBS) is caused by a microdeletion of chromosome arm 7q11.23. A rapid and inexpensive genotyping method to detect microdeletion on 7q11.23 needs to be developed for the diagnosis of WBS. This study describes the development of a new type of molecular diagnosis method to detect microdeletion on 7q11.23 based upon high-resolution melting (HRM). Four genes on 7q11.23 were selected as the target genes for the deletion genotyping. dNTP-limited duplex PCR was used to amplify the reference gene, CFTR, and one of the four genes respectively on 7q11.23. An HRM assay was performed on the PCR products, and the height ratio of the negative derivative peaks between the target gene and reference gene was employed to analyze the copy number variation of the target region. A new genotyping method for detecting 7q11.23 deletion was developed based upon dNTP-limited PCR and HRM, which cost only 96 min. Samples from 15 WBS patients and 12 healthy individuals were genotyped by this method in a blinded fashion, and the sensitivity and specificity was 100% (95% CI, 0.80-1, and 95% CI, 0.75-1, respectively) which was proved by CytoScan HD array. The HRM assay we developed is an rapid, inexpensive, and highly accurate method for genotyping 7q11.23 deletion. It is potentially useful in the clinical diagnosis of WBS. Copyright © 2018 Elsevier B.V. All rights reserved.

  4. William Barlow and the Determination of Atomic Arrangement in Crystals.

    PubMed

    Mauskopf, Seymour H

    2015-04-01

    William Barlow (1845-1934) was an important if unconventional scientist, known for having developed the 'closest-packing' atomic models of crystal structure. He resumed an early nineteenth-century tradition of utilizing crystallographical and chemical data to determine atomic arrangements in crystals. This essay recounts Barlow's career and scientific activity in three parts: (a) His place in the tradition of determining atomic arrangement in context of this earlier tradition and of contemporaneous developments of crystallography and chemistry, (b) his unconventional career, and (c) the 'success' of his program to determine atomic arrangements in crystals and its influence on the work of William Lawrence Bragg.

  5. Autistic disorder in patients with Williams-Beuren syndrome: a reconsideration of the Williams-Beuren syndrome phenotype.

    PubMed

    Tordjman, Sylvie; Anderson, George M; Botbol, Michel; Toutain, Annick; Sarda, Pierre; Carlier, Michèle; Saugier-Veber, Pascale; Baumann, Clarisse; Cohen, David; Lagneaux, Céline; Tabet, Anne-Claude; Verloes, Alain

    2012-01-01

    Williams-Beuren syndrome (WBS), a rare developmental disorder caused by deletion of contiguous genes at 7q11.23, has been characterized by strengths in socialization (overfriendliness) and communication (excessive talkativeness). WBS has been often considered as the polar opposite behavioral phenotype to autism. Our objective was to better understand the range of phenotypic expression in WBS and the relationship between WBS and autistic disorder. The study was conducted on 9 French individuals aged from 4 to 37 years old with autistic disorder associated with WBS. Behavioral assessments were performed using Autism Diagnostic Interview-Revised (ADI-R) and Autism Diagnostic Observation Schedule (ADOS) scales. Molecular characterization of the WBS critical region was performed by FISH. FISH analysis indicated that all 9 patients displayed the common WBS deletion. All 9 patients met ADI-R and ADOS diagnostic criteria for autism, displaying stereotypies and severe impairments in social interaction and communication (including the absence of expressive language). Additionally, patients showed improvement in social communication over time. The results indicate that comorbid autism and WBS is more frequent than expected and suggest that the common WBS deletion can result in a continuum of social communication impairment, ranging from excessive talkativeness and overfriendliness to absence of verbal language and poor social relationships. Appreciation of the possible co-occurrence of WBS and autism challenges the common view that WBS represents the opposite behavioral phenotype of autism, and might lead to improved recognition of WBS in individuals diagnosed with autism.

  6. The proceedings of the 15th professional conference on Williams Syndrome.

    PubMed

    Walton, Jennifer R; Martens, Marilee A; Pober, Barbara R

    2017-05-01

    Williams Syndrome (WS) is a contiguous gene deletion disorder, caused by the deletion of approximately 26-28 genes from chromosome 7 (7q11.23). Individuals with WS have complex medical, developmental, and behavioral features, requiring multidisciplinary and interdisciplinary collaboration. Guidelines detailing the identification, evaluation, and monitoring of individuals with WS need clarification, especially for primary care providers who are first-line in their management. This report summarizes the proceedings of the 2016 Professional Conference on WS in Columbus, OH. Presentations were directed towards primary care providers and subspecialists, emphasizing evidence-based practices for treating the prevalent medical and behavioral features of WS. Included in this report are findings from a panel of cardiovascular experts discussing three case studies on treatment of hypertension and the use of sedation or anesthesia for non-cardiac procedures. Abstracts from individual expert presenters are included, covering various medical and behavioral topics, and providing updates in management of WS individuals. The following topics were discussed: differences in phenotypes of 7q11.23 deletion versus duplication, growth parameters, endocrine concerns, sleep difficulties, behaviors to monitor, and pharmacological options, the neurodevelopmental profile of WS individuals, and the importance of monitoring medical and behavioral concerns as WS individuals transition to adulthood. © 2017 Wiley Periodicals, Inc.

  7. 77 FR 65623 - Security Zones; USCGC WILLIAM FLORES Commissioning Ceremony, Ybor Channel; Tampa, FL

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-10-30

    ...-AA87 Security Zones; USCGC WILLIAM FLORES Commissioning Ceremony, Ybor Channel; Tampa, FL AGENCY: Coast... zone on the waters of Ybor Channel in Tampa, Florida around the USCGC WILLIAM FLORES immediately before... FLORES, official parties, dignitaries, the public, and surrounding waterways from terrorist acts...

  8. 77 FR 63308 - J. William Foley Incorporated v. United Illuminating Company; Notice of Complaint

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-10-16

    ... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Docket No. EL12-106-000] J. William Foley Incorporated v. United Illuminating Company; Notice of Complaint Take notice that on October 5...) and 825(e), J. William Foley Incorporated (Complainant) filed a formal complaint against United...

  9. Increased overall cortical connectivity with syndrome specific local decreases suggested by atypical sleep-EEG synchronization in Williams syndrome.

    PubMed

    Gombos, Ferenc; Bódizs, Róbert; Kovács, Ilona

    2017-07-21

    Williams syndrome (7q11.23 microdeletion) is characterized by specific alterations in neurocognitive architecture and functioning, as well as disordered sleep. Here we analyze the region, sleep state and frequency-specific EEG synchronization of whole night sleep recordings of 21 Williams syndrome and 21 typically developing age- and gender-matched subjects by calculating weighted phase lag indexes. We found broadband increases in inter- and intrahemispheric neural connectivity for both NREM and REM sleep EEG of Williams syndrome subjects. These effects consisted of increased theta, high sigma, and beta/low gamma synchronization, whereas alpha synchronization was characterized by a peculiar Williams syndrome-specific decrease during NREM states (intra- and interhemispheric centro-temporal) and REM phases of sleep (occipital intra-area synchronization). We also found a decrease in short range, occipital connectivity of NREM sleep EEG theta activity. The striking increased overall synchronization of sleep EEG in Williams syndrome subjects is consistent with the recently reported increase in synaptic and dendritic density in stem-cell based Williams syndrome models, whereas decreased alpha and occipital connectivity might reflect and underpin the altered microarchitecture of primary visual cortex and disordered visuospatial functioning of Williams syndrome subjects.

  10. Dr. Caleb Williams Saleeby: The Complete Eugenicist.

    ERIC Educational Resources Information Center

    Rodwell, Grant

    1997-01-01

    Profiles the work of Dr. Caleb Williams Saleeby, a late 19th-century propagandist for eugenics. Eugenics is a science that deals with the transmission of hereditary racial traits, coupled with a desire to use this for the elimination of social ills. Discusses Saleeby's work with the Eugenics Education Society. (MJP)

  11. Water resources of Prince William Forest Park, Virginia

    USGS Publications Warehouse

    Brown, G. Allan

    1981-01-01

    Prince William Forest Park is in the southern part of Prince William County, Va. Its natural beauty and nearness to Washington, D.C. have made it one of the most popular recreation areas in northern Virginia. To help the National Park Service plan the development of new facilities and effectively manage its total resources, the U.S. Geological Survey made a hydrologic study of the park from October 1972 to November 1975. The overall objective of the Survey 's study was to evalute the quantity and quality of the park 's water resources. Available information was compiled and analyzed, and new data on streamflow, ground-water levels, and the chemical quality of water were collected. Test wells were drilled and aquifer tests made at the sites of wells to evaluate the occurrence and availability of ground water. (USGS)

  12. The Cosmology of William Herschel

    NASA Astrophysics Data System (ADS)

    Hoskin, M.

    2009-08-01

    William Herschel was an amateur astronomer for half his life, until his discovery of Uranus earned him a royal pension. He then set himself to study "the construction of the heavens" with great reflectors, and discovered over 2,500 nebulae and star clusters. Clusters had clearly formed by the action of gravity, and so scattered clusters would in time become ever more compressed: scattered clusters were young, compressed clusters old. This marked the end of the 'clockwork' universe of Newton and Leibniz.

  13. 77 FR 58819 - Notice of Submission for OMB Review; Federal Student Aid; William D. Ford Federal Direct Loan...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-09-24

    ... DEPARTMENT OF EDUCATION Notice of Submission for OMB Review; Federal Student Aid; William D. Ford... William D. Ford Federal Direct Loan Program will use this form to request forbearance on their loans when... of Collection: William D. Ford Federal Direct Loan Program General Forbearance Request. OMB Control...

  14. Clinical utility of an array comparative genomic hybridization analysis for Williams syndrome.

    PubMed

    Yagihashi, Tatsuhiko; Torii, Chiharu; Takahashi, Reiko; Omori, Mikimasa; Kosaki, Rika; Yoshihashi, Hiroshi; Ihara, Masahiro; Minagawa-Kawai, Yasuyo; Yamamoto, Junichi; Takahashi, Takao; Kosaki, Kenjiro

    2014-11-01

    To reveal the relation between intellectual disability and the deleted intervals in Williams syndrome, we performed an array comparative genomic hybridization analysis and standardized developmental testing for 11 patients diagnosed as having Williams syndrome based on fluorescent in situ hybridization testing. One patient had a large 4.2-Mb deletion spanning distally beyond the common 1.5-Mb intervals observed in 10/11 patients. We formulated a linear equation describing the developmental age of the 10 patients with the common deletion; the developmental age of the patient with the 4.2-Mb deletion was significantly below the expectation (developmental age = 0.51 × chronological age). The large deletion may account for the severe intellectual disability; therefore, the use of array comparative genomic hybridization may provide practical information regarding individuals with Williams syndrome. © 2014 Japanese Teratology Society.

  15. 78 FR 77508 - Duke Energy Carolinas, LLC; William States Lee III Nuclear Station, Units 1 and 2; Combined...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-12-23

    ..., LLC; William States Lee III Nuclear Station, Units 1 and 2; Combined Licenses Application Review... Environmental Impact Statement [EIS] for Combined Licenses (COLs) for William States Lee III Nuclear Station... be accessed online at the NRC's William States Lee III Nuclear Station--specific Web page at: www.nrc...

  16. Williams with TVIS hardware in Zvezda Service module

    NASA Image and Video Library

    2007-02-26

    ISS014-E-15136 (26 Feb. 2007) --- Astronaut Sunita L. Williams, Expedition 14 flight engineer, performs maintenance work on the Treadmill Vibration Isolation System (TVIS) during routine in-flight maintenance (IFM) in the Zvezda Service Module of the International Space Station.

  17. Sir William Jenner (1815-1898) and Queen Victoria.

    PubMed

    Seddon, David; Queen Victoria

    2004-08-01

    This biographical note outlines the work of Sir William Jenner (1815-1898), court physician 1861-93 and President of the Royal College of Physicians 1881-8. He was the first to distinguish between typhoid and typhus both clinically and pathologically.

  18. STS-79 Commander William Readdy in White Room

    NASA Technical Reports Server (NTRS)

    1996-01-01

    STS-79 Commander William F. Readdy gets ready to climb into the flight deck of the Space Shuttle Atlantis at Launch Pad 39A. Assisting him are white room closeout crew members Travis Thompson (from left), Jean Alexander and Jim Davis.

  19. Brief Report: Developing Spatial Frequency Biases for Face Recognition in Autism and Williams Syndrome

    ERIC Educational Resources Information Center

    Leonard, Hayley C.; Annaz, Dagmara; Karmiloff-Smith, Annette; Johnson, Mark H.

    2011-01-01

    The current study investigated whether contrasting face recognition abilities in autism and Williams syndrome could be explained by different spatial frequency biases over developmental time. Typically-developing children and groups with Williams syndrome and autism were asked to recognise faces in which low, middle and high spatial frequency…

  20. Denigrating Carl Rogers: William Coulson's Last Crusade.

    ERIC Educational Resources Information Center

    Kirschenbaum, Howard

    1991-01-01

    Reviews William Coulson's assertions that Carl Rogers, Abraham Maslow, and he initiated the humanistic education field, that Rogers repudiated his philosophy late in life, and that they owe the nation's parents an apology. Argues that these charges are groundless and provides examples and quotations from Rogers' later writings to show how Rogers…

  1. Epistemology in Excess? A Response to Williams

    ERIC Educational Resources Information Center

    Siegei, Harvey

    2017-01-01

    Emma Williams' "In Excess of Epistemology" admirably endeavours to open the way to an account of critical thinking that goes beyond the one I have defended ad nauseum in recent decades by developing, via the work of Charles Taylor and Martin Heidegger, "a radically different conception of thinking and the human being who…

  2. Fears, Hyperacusis and Musicality in Williams Syndrome

    ERIC Educational Resources Information Center

    Blomberg, Stefan; Rosander, Michael; Andersson, Gerhard

    2006-01-01

    The study investigated the prevalence of fear and hyperacusis and the possible connections between fear, hyperacusis and musicality in a Swedish sample of individuals with Williams syndrome (WS). The study included 38 individuals and a cross-sectional design, with no matched control group. Two persons, who knew the participant well, completed a…

  3. Executive Functions in Individuals with Williams Syndrome

    ERIC Educational Resources Information Center

    Menghini, D.; Addona, F.; Costanzo, F.; Vicari, S.

    2010-01-01

    Background: The present study was aimed at investigating working memory (WM) and executive functions capacities in individuals with Williams syndrome (WS) as compared with mental-age matched typically developing (TD) children. Method: In order to serve the study goal, a sizeable battery of tasks tapping WM as well as attention, memory, planning,…

  4. Williams working in the US Lab during Expedition 15

    NASA Image and Video Library

    2007-06-01

    ISS015-E-10579 (1 June 2007) --- Astronaut Sunita L. Williams, Expedition 15 flight engineer, wears communication headgear while using a computer in the Destiny laboratory of the International Space Station.

  5. Intelligence in Williams Syndrome Is Related to STX1A, Which Encodes a Component of the Presynaptic SNARE Complex

    PubMed Central

    Gao, Michael C.; Bellugi, Ursula; Dai, Li; Mills, Debra L.; Sobel, Eric M.; Lange, Kenneth; Korenberg, Julie R.

    2010-01-01

    Although genetics is the most significant known determinant of human intelligence, specific gene contributions remain largely unknown. To accelerate understanding in this area, we have taken a new approach by studying the relationship between quantitative gene expression and intelligence in a cohort of 65 patients with Williams Syndrome (WS), a neurodevelopmental disorder caused by a 1.5 Mb deletion on chromosome 7q11.23. We find that variation in the transcript levels of the brain gene STX1A correlates significantly with intelligence in WS patients measured by principal component analysis (PCA) of standardized WAIS-R subtests, r  = 0.40 (Pearson correlation, Bonferroni corrected p-value  = 0.007), accounting for 15.6% of the cognitive variation. These results suggest that syntaxin 1A, a neuronal regulator of presynaptic vesicle release, may play a role in WS and be a component of the cellular pathway determining human intelligence. PMID:20422020

  6. STS-118 Astronaut Williams and Expedition 15 Engineer Anderson Perform EVA

    NASA Technical Reports Server (NTRS)

    2007-01-01

    As the construction continued on the International Space Station (ISS), STS-118 Astronaut Dave Williams, representing the Canadian Space Agency, participated in the fourth and final session of Extra Vehicular Activity (EVA). During the 5 hour space walk, Williams and Expedition 15 engineer Clay Anderson (out of frame) installed the External Wireless Instrumentation System Antenna, attached a stand for the shuttle robotic arm extension boom, and retrieved the two Materials International Space Station Experiments (MISSE) for return to Earth. MISSE collects information on how different materials weather in the environment of space.

  7. William T. Grant Foundation 2012 Annual Report

    ERIC Educational Resources Information Center

    William T. Grant Foundation, 2013

    2013-01-01

    The William T. Grant Foundation was founded in 1936 to support strong social science research focused on "the enrichment of life, with a primary interest in people and in their adjustment to the world in which they live." Today, its funding priorities reflect this interest in research that affects young people's lives. This Annual Report…

  8. Development of Verbal Passive in Williams Syndrome

    ERIC Educational Resources Information Center

    Perovic, Alexandra; Wexler, Kenneth

    2010-01-01

    Purpose: To experimentally investigate knowledge of passives of actional ("hold") and psychological ("love") verbs in children with Williams syndrome (WS). Passives are usually reported to be in line with mental age in WS. However, studies usually focus on passives of actional verbs only. Method: Twenty-six children with WS, ages 6-16, and 3…

  9. Herbert Hoover and William Shakespeare: Two Writers on Ethics.

    ERIC Educational Resources Information Center

    Somers, Margaret L.

    1979-01-01

    Outlines the ways in which Herbert Hoover and William Shakespeare wrote about professional ethics (for engineers and kings, respectively) using the writing techniques of concreteness, audience awareness, and development by induction. (TJ)

  10. Williams in the U.S. Laboratory during Expedition 13

    NASA Image and Video Library

    2006-08-22

    ISS013-E-70806 (22 Aug. 2006) --- Astronaut Jeffrey N. Williams, Expedition 13 NASA space station science officer and flight engineer, uses a computer in the Destiny laboratory of the International Space Station.

  11. New TFII-I family target genes involved in embryonic development.

    PubMed

    Makeyev, Aleksandr V; Bayarsaihan, Dashzeveg

    2009-09-04

    Two members of the TFII-I family transcription factor genes, GTF2I and GTF2IRD1, are the prime candidates responsible for the craniofacial and cognitive abnormalities of Williams syndrome patients. We have previously generated mouse lines with targeted disruption of Gtf2i and Gtf2ird1. Microarray analysis revealed significant changes in the expression profile of mutant embryos. Here we described three unknown genes that were dramatically down-regulated in mutants. The 2410018M08Rik/Scand3 gene encodes a protein of unknown function with CHCH and hATC domains. Scand3 is down-regulated during mouse embryonic stem cell (ES) differentiation. 4933436H12Rik is a testis-specific gene, which encodes a protein with no known domains. It is expressed in mouse ES cells. 1110008P08Rik/Kbtbd7 encodes an adapter protein with BTB/POZ, BACK, and Kelch motifs, previously shown to recruit substrates to the enzymatic complexes of the histone modifying or E3 ubiquitin ligase activities. Based on its expression pattern Kbtbd7 may have a specific role in brain development and function. All three genes possess well-conserved TFII-I-binding consensus sites within proximal promoters. Therefore our analysis suggests that these genes can be direct targets of TFII-I proteins and their impaired expression, as a result of the GTF2I and GTF2IRD1 haploinsufficiency, could contribute to the etiology of Williams syndrome.

  12. Psycholinguistic Abilities of Children with Williams Syndrome

    ERIC Educational Resources Information Center

    Rossi, Natalia F.; Heinze, Elena Garayzabal; Giacheti, Celia M.; Goncalves, Oscar F.; Sampaio, Adriana

    2012-01-01

    The objective of this study was to investigate the psycholinguistic abilities of children with Williams syndrome (WS) and typically developing children using the Illinois Test of Psycholinguistic Abilities (ITPA). Performance on the ITPA was analysed in a group with WS (N=20, mean age=8.5 years, SD=1.62) and two typically developing groups,…

  13. Learning without Awareness Revisited: Extending Williams (2005)

    ERIC Educational Resources Information Center

    Hama, Mika; Leow, Ronald P.

    2010-01-01

    The role of awareness or consciousness in learning has been a relatively contentious issue in non-SLA fields (e.g., cognitive psychology). With the publications of Williams (2004, 2005), a similar debate appears to be brewing in the field of SLA. Contrary to Leow (2000), who reported that unawareness did not appear to play an important role in…

  14. The Brief Military Career of Dr. William H. Welch.

    PubMed

    Gilman, James K

    2017-03-01

    The purpose of this article is to examine the Army service of Dr. William H. Welch during World War I. Archival research utilizing prime source documents in the William H. Welch Collection of the Alan M. Chesney Medical Archives for the Johns Hopkins Medical Institutions. Welch joined the Army at the age of 67 after serving as one of the principal transformational forces for reforming medical education in the United States and founding the first academic institution for educating public health professionals in the United States, the Johns Hopkins School of Public Health and Hygiene. His longstanding relationship with Army Surgeon General William Gorgas served as the backdrop for Welch's service. Welch served as both a staff officer and as a traveling medical inspector general, assessing the medical care of troops preparing for overseas duty. He did not adapt particularly well to military dress and decorum but his status as one of the icons of American medicine rendered these shortcomings insignificant. Welch was joined in Army service by a number of American medical luminaries-both Mayo brothers, George Crile, and Harvey Cushing among them. Although Welch remained on active duty for only 13 months, he maintained a nominal relationship with Army medicine through appointment to the Medical Officer Reserve Corps until the time of his death. 2016 marks the centennial of the establishment of the first independent academic institution in America dedicated to education and training of professionals focused on public health and hygiene issues. 2017 marks the centennial of U.S. entry into World War I. Dr. William H. Welch played an important role in both of these historic events and, although his active service was brief, the impact of his example was substantial. Analysis of his military career in its full historical context provides insight into the relationship between academic medicine and military medicine during periods of armed conflict. Reprint & Copyright © 2017

  15. 78 FR 52169 - Agency Information Collection Activities; Comment Request; William D. Ford Federal Direct Loan...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-08-22

    ... DEPARTMENT OF EDUCATION [Docket No. ED-2013-ICCD-0109] Agency Information Collection Activities; Comment Request; William D. Ford Federal Direct Loan (Direct Loan) Program Federal Direct PLUS Loan Master... to this notice will be considered public records. Title of Collection: William D. Ford Federal Direct...

  16. Noguchi and Williams in hatch area during Expedition 22

    NASA Image and Video Library

    2010-01-03

    ISS022-E-018820 (3 Jan. 2010) --- NASA astronaut Jeffrey Williams (right), Expedition 22 commander; and Japan Aerospace Exploration Agency astronaut Soichi Noguchi, flight engineer, are pictured in a hatch on the International Space Station.

  17. Autistic Disorder in Patients with Williams-Beuren Syndrome: A Reconsideration of the Williams-Beuren Syndrome Phenotype

    PubMed Central

    Tordjman, Sylvie; Anderson, George M.; Botbol, Michel; Toutain, Annick; Sarda, Pierre; Carlier, Michèle; Saugier-Veber, Pascale; Baumann, Clarisse; Cohen, David; Lagneaux, Céline; Tabet, Anne-Claude; Verloes, Alain

    2012-01-01

    Background Williams-Beuren syndrome (WBS), a rare developmental disorder caused by deletion of contiguous genes at 7q11.23, has been characterized by strengths in socialization (overfriendliness) and communication (excessive talkativeness). WBS has been often considered as the polar opposite behavioral phenotype to autism. Our objective was to better understand the range of phenotypic expression in WBS and the relationship between WBS and autistic disorder. Methodology The study was conducted on 9 French individuals aged from 4 to 37 years old with autistic disorder associated with WBS. Behavioral assessments were performed using Autism Diagnostic Interview-Revised (ADI-R) and Autism Diagnostic Observation Schedule (ADOS) scales. Molecular characterization of the WBS critical region was performed by FISH. Findings FISH analysis indicated that all 9 patients displayed the common WBS deletion. All 9 patients met ADI-R and ADOS diagnostic criteria for autism, displaying stereotypies and severe impairments in social interaction and communication (including the absence of expressive language). Additionally, patients showed improvement in social communication over time. Conclusions The results indicate that comorbid autism and WBS is more frequent than expected and suggest that the common WBS deletion can result in a continuum of social communication impairment, ranging from excessive talkativeness and overfriendliness to absence of verbal language and poor social relationships. Appreciation of the possible co-occurrence of WBS and autism challenges the common view that WBS represents the opposite behavioral phenotype of autism, and might lead to improved recognition of WBS in individuals diagnosed with autism. PMID:22412832

  18. Experiences of Bullying for Individuals with Williams Syndrome

    ERIC Educational Resources Information Center

    Fisher, Marisa H.; Lough, Emma; Griffin, Megan M.; Lane, Laurel A.

    2017-01-01

    Background: Individuals with intellectual disability experience high rates of bullying, but it is not known how people with specific syndromes, such as Williams syndrome (WS), experience and respond to bullying. Given their behavioral profile, including hypersociability and heightened anxiety, and their risk for experiencing other forms of…

  19. Williams with Mission Patches in the A/L

    NASA Image and Video Library

    2016-08-14

    Commander Jeff Williams poses for a photo in the Quest Airlock (A/L) with all of his mission patches. The patches are, from left, STS-101, Soyuz TMA-8, Expedition 13, Soyuz TMA-16, Expedition 21, Expedition 22, Soyuz TMA-20M, Expedition 47, and Expedition 48.

  20. Junior Leader Innovation: William Sims & Continuous Aim Fire

    DTIC Science & Technology

    2012-06-06

    construction ever perpetrated by the white race.” – LT William S. Sims Admiral Sir Percy Scott , Royal Navy...Admiral Mason, Chief, Bureau of Ordnance Ridley McLean “He continued to be a thorn in the fat flesh of the naval hierarchy during his entire

  1. Marginalizing Women: Images of Pregnancy in Williams Obstetrics

    PubMed Central

    Smith, Sheila A.; Condit, Deirdre M.

    2000-01-01

    This research analyzes the historical development of the medical construction of the pregnant body in 17 of 20 editions of Williams Obstetrics, an obstetrical textbook published continually from 1904 to 1997. Examination of the visual imagery of these works produced three key findings. First, depictions of the healthy or “normal” pregnant body are virtually absent throughout the series. Second, visual depictions of women's full bodies adhere to a race-based hierarchy of presentation. Finally, the fundamental discourse about pregnant and female bodies communicated to physicians (primarily) by these images is one of pathology and fragmentation. We conclude that the resulting social and medical construction of the pregnant and female body presented in the Williams series is one of disembodiment, abjection, and ultimately marginality. These findings support recent feminist research that criticizes both the increasing erasure of the person of the women from the medical interpretation of pregnancy and the concomitant decrease in women's perceived sense of empowerment as pregnant beings. PMID:17273202

  2. Commerical Crew Astronaut Suni Williams in SpaceX's Spacesuit

    NASA Image and Video Library

    2018-05-17

    NASA Astronaut Suni Williams, fully suited in SpaceX’s spacesuit, interfaces with the display inside a mock-up of the Crew Dragon spacecraft in Hawthorne, California, during a testing exercise on Tuesday, April 3, 2018.

  3. 3. Historic American Buildings Survey William C. Everhart, Photographer October ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    3. Historic American Buildings Survey William C. Everhart, Photographer October 1958 FRONT and EAST SIDE ELEVATIONS - Sacred Heart Mission, Interstate 90 & Interchange 39, Cataldo, Shoshone County, ID

  4. 6. Historic American Buildings Survey William C. Everhart, Photographer October ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    6. Historic American Buildings Survey William C. Everhart, Photographer October 1958 FRONT and WEST SIDE ELEVATIONS - Sacred Heart Mission, Interstate 90 & Interchange 39, Cataldo, Shoshone County, ID

  5. William Herschel and Comets

    NASA Astrophysics Data System (ADS)

    Sullivan, Woodruff

    2018-01-01

    I examine the observational and theoretical researches of William Herschel on 21 comets that he observed over the period 1781 to 1812. Herschel's focus, unlike most contemporaries, was on their physical structure, not their orbits. He forged a strong connection between comets and his nebulae with a scheme of cometary "maturation" (1812) involved a comet traveling from star to star after its central "planetary body'; was born from gravitational collapse of a nebula. During close passages of a star, the comet brightened and lost mass from its atmosphere; at other times, when between stars, it encountered nebulae and was rejuvenated by picking up more mass. Laplace soon adopted these ideas to improve his nebula hypothesis for solar system formation.

  6. Anxiety and Repetitive Behaviours in Autism Spectrum Disorders and Williams Syndrome: A Cross-Syndrome Comparison

    ERIC Educational Resources Information Center

    Rodgers, Jacqui; Riby, Deborah M.; Janes, Emily; Connolly, Brenda; McConachie, Helen

    2012-01-01

    Children with Autism Spectrum Disorder or Williams syndrome are vulnerable to anxiety. The factors that contribute to this risk remain unclear. This study compared anxiety in autism spectrum disorder and Williams Syndrome and examined the relationship between repetitive behaviours and anxiety. Thirty-four children with autism and twenty children…

  7. 34 CFR 662.22 - How does the J. William Fulbright Foreign Scholarship Board select fellows?

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 34 Education 3 2013-07-01 2013-07-01 false How does the J. William Fulbright Foreign Scholarship Board select fellows? 662.22 Section 662.22 Education Regulations of the Offices of the Department of... Foreign Scholarship Board select fellows? (a) The J. William Fulbright Foreign Scholarship Board selects...

  8. 34 CFR 662.22 - How does the J. William Fulbright Foreign Scholarship Board select fellows?

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 34 Education 3 2010-07-01 2010-07-01 false How does the J. William Fulbright Foreign Scholarship Board select fellows? 662.22 Section 662.22 Education Regulations of the Offices of the Department of... Foreign Scholarship Board select fellows? (a) The J. William Fulbright Foreign Scholarship Board selects...

  9. 34 CFR 662.22 - How does the J. William Fulbright Foreign Scholarship Board select fellows?

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 34 Education 3 2014-07-01 2014-07-01 false How does the J. William Fulbright Foreign Scholarship Board select fellows? 662.22 Section 662.22 Education Regulations of the Offices of the Department of... Foreign Scholarship Board select fellows? (a) The J. William Fulbright Foreign Scholarship Board selects...

  10. 34 CFR 662.22 - How does the J. William Fulbright Foreign Scholarship Board select fellows?

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 34 Education 3 2012-07-01 2012-07-01 false How does the J. William Fulbright Foreign Scholarship Board select fellows? 662.22 Section 662.22 Education Regulations of the Offices of the Department of... Foreign Scholarship Board select fellows? (a) The J. William Fulbright Foreign Scholarship Board selects...

  11. 34 CFR 662.22 - How does the J. William Fulbright Foreign Scholarship Board select fellows?

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 34 Education 3 2011-07-01 2011-07-01 false How does the J. William Fulbright Foreign Scholarship Board select fellows? 662.22 Section 662.22 Education Regulations of the Offices of the Department of... Foreign Scholarship Board select fellows? (a) The J. William Fulbright Foreign Scholarship Board selects...

  12. Williams works at the MSG during Expedition 13

    NASA Image and Video Library

    2006-05-04

    ISS013-E-14536 (4 May 2006) --- Astronaut Jeffrey N. Williams, Expedition 13 NASA space station science officer and flight engineer, conducts the first run of the Pore Formation and Mobility Investigation (PFMI) in the Microgravity Science Glovebox (MSG) in the Destiny laboratory of the International Space Station.

  13. Williams works at the MSG during Expedition 13

    NASA Image and Video Library

    2006-05-04

    ISS013-E-14573 (4 May 2006) --- Astronaut Jeffrey N. Williams, Expedition 13 NASA space station science officer and flight engineer, conducts the first run of the Pore Formation and Mobility Investigation (PFMI) in the Microgravity Science Glovebox (MSG) in the Destiny laboratory of the International Space Station.

  14. Williams works at the MSG during Expedition 13

    NASA Image and Video Library

    2006-05-04

    ISS013-E-14524 (4 May 2006) --- Astronaut Jeffrey N. Williams, Expedition 13 NASA space station science officer and flight engineer, conducts the first run of the Pore Formation and Mobility Investigation (PFMI) in the Microgravity Science Glovebox (MSG) in the Destiny laboratory of the International Space Station.

  15. Williams works at the MSG during Expedition 13

    NASA Image and Video Library

    2006-05-04

    ISS013-E-14537 (4 May 2006) --- Astronaut Jeffrey N. Williams, Expedition 13 NASA space station science officer and flight engineer, conducts the first run of the Pore Formation and Mobility Investigation (PFMI) in the Microgravity Science Glovebox (MSG) in the Destiny laboratory of the International Space Station.

  16. Williams works at the MSG during Expedition 13

    NASA Image and Video Library

    2006-05-04

    ISS013-E-14531 (4 May 2006) --- Astronaut Jeffrey N. Williams, Expedition 13 NASA space station science officer and flight engineer, conducts the first run of the Pore Formation and Mobility Investigation (PFMI) in the Microgravity Science Glovebox (MSG) in the Destiny laboratory of the International Space Station.

  17. Electrophysiological Correlates of Semantic Processing in Williams Syndrome

    ERIC Educational Resources Information Center

    Pinheiro, Ana P.; Galdo-Alvarez, Santaigo; Sampaio, Adriana; Niznikiewicz, Margaret; Goncalves, Oscar F.

    2010-01-01

    Williams syndrome (WS), a genetic neurodevelopmental disorder due to microdeletion in chromosome 7, has been described as a syndrome with an intriguing socio-cognitive phenotype. Cognitively, the relative preservation of language and face processing abilities coexists with severe deficits in visual-spatial tasks, as well as in tasks involving…

  18. 7. William E. Barrett, Photographer, August 1975. LOG PONDS LOOKING ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    7. William E. Barrett, Photographer, August 1975. LOG PONDS LOOKING WEST FROM POWERHOUSE ROOF. TRANSFORMER SHED IN FOREGROUND. - Meadow River Lumber Company, Highway 60, Rainelle, Greenbrier County, WV

  19. STS-34 Commander Williams in KSC O and C Bldg prepares for TCDT activities

    NASA Technical Reports Server (NTRS)

    1989-01-01

    STS-34 Commander Donald E. Williams is assisted by technicians with donning his launch and entry suit (LES) in the Kennedy Space Center (KSC) Operations and Checkout (O and C) Building prior to leaving for Launch Complex (LC) Pad 39B during terminal countdown demonstration test (TCDT) activities. Williams also catches a few minutes of relaxation while reclining in his chair.

  20. Long-term outcome of large artificial patch aortic repair for diffuse stenosis in Williams syndrome.

    PubMed

    Sughimoto, Koichi; Takahara, Yoshiharu; Mogi, Kenji; Sakurai, Manabu; Aoki, Chikashi

    2010-10-01

    There have been only a few reports concerning the long-term results of a surgical procedure using a large artificial patch for patients with Williams syndrome. Twelve years have passed since a patient with William's syndrome underwent a surgery with a patch angioplasty for the diffuse supravalvular aortic stenosis and deformities of the neck branch arteries. The patient had a well-balanced aortic growth without stenotic or aneurysmal changes, which was confirmed during the time of the second surgery when replacing the mitral valve. This technique of using a large patch has proven to be safe for Williams syndrome patients with diffuse supravalvular aortic stenosis in the long term.

  1. Speculation on Curriculum from the Perspective of William James.

    ERIC Educational Resources Information Center

    Shubert, William H; Zissis, Georgiana

    1988-01-01

    This article discusses the implications for curriculum theory, research, and practice of William James' thought. Also considered is the question of what curriculum theory and research might be like if James had garnered greater influence than Thorndike. (IAH)

  2. 34 CFR 663.22 - How does the J. William Fulbright Foreign Scholarship Board select fellows?

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 34 Education 3 2013-07-01 2013-07-01 false How does the J. William Fulbright Foreign Scholarship Board select fellows? 663.22 Section 663.22 Education Regulations of the Offices of the Department of... Scholarship Board select fellows? The J. William Fulbright Foreign Scholarship Board selects fellows on the...

  3. 34 CFR 663.22 - How does the J. William Fulbright Foreign Scholarship Board select fellows?

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 34 Education 3 2014-07-01 2014-07-01 false How does the J. William Fulbright Foreign Scholarship Board select fellows? 663.22 Section 663.22 Education Regulations of the Offices of the Department of... Scholarship Board select fellows? The J. William Fulbright Foreign Scholarship Board selects fellows on the...

  4. 34 CFR 663.22 - How does the J. William Fulbright Foreign Scholarship Board select fellows?

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 34 Education 3 2010-07-01 2010-07-01 false How does the J. William Fulbright Foreign Scholarship Board select fellows? 663.22 Section 663.22 Education Regulations of the Offices of the Department of... Scholarship Board select fellows? The J. William Fulbright Foreign Scholarship Board selects fellows on the...

  5. 34 CFR 663.22 - How does the J. William Fulbright Foreign Scholarship Board select fellows?

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 34 Education 3 2011-07-01 2011-07-01 false How does the J. William Fulbright Foreign Scholarship Board select fellows? 663.22 Section 663.22 Education Regulations of the Offices of the Department of... Scholarship Board select fellows? The J. William Fulbright Foreign Scholarship Board selects fellows on the...

  6. 34 CFR 663.22 - How does the J. William Fulbright Foreign Scholarship Board select fellows?

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 34 Education 3 2012-07-01 2012-07-01 false How does the J. William Fulbright Foreign Scholarship Board select fellows? 663.22 Section 663.22 Education Regulations of the Offices of the Department of... Scholarship Board select fellows? The J. William Fulbright Foreign Scholarship Board selects fellows on the...

  7. 22. William E. Barrett, Photographer, August 1975. CHIPPER OR 'HOG' ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    22. William E. Barrett, Photographer, August 1975. CHIPPER OR 'HOG' FOR REDUCING SCRAPS TO WOOD CHIPS. HOUSING PARTIALLY REMOVED. - Meadow River Lumber Company, Highway 60, Rainelle, Greenbrier County, WV

  8. 33 CFR 167.1701 - In Prince William Sound: Precautionary areas.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... HOMELAND SECURITY (CONTINUED) PORTS AND WATERWAYS SAFETY OFFSHORE TRAFFIC SEPARATION SCHEMES Description of Traffic Separation Schemes and Precautionary Areas Pacific West Coast § 167.1701 In Prince William Sound...

  9. 76 FR 79228 - Combined Licenses at William States Lee III Nuclear Station Site, Units 1 and 2; Duke Energy...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-12-21

    ... William States Lee III Nuclear Station Site, Units 1 and 2; Duke Energy Carolinas, LLC AGENCY: Nuclear... Statement for Combined Licenses (COL) for William States Lee III Nuclear Station Units 1 and 2 [Lee Nuclear... draft EIS can be accessed online at the NRC's William States Lee III Nuclear Site Web page at http://www...

  10. Adaptive Functioning in Williams Syndrome: A Systematic Review

    ERIC Educational Resources Information Center

    Brawn, Gabrielle; Porter, Melanie

    2018-01-01

    Literature on the level of adaptive functioning and relative strengths and weaknesses in functioning of individuals with Williams syndrome (WS) was reviewed. The electronic databases PsycINFO, PubMed, Expanded Academic, Web of Science, Scopus and ProQuest were searched electronically for relevant articles and dissertations using the search terms…

  11. 77 FR 47374 - Notice of Proposed Information Collection Requests; Federal Student Aid; William D. Ford Federal...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-08-08

    ... which borrowers in the William D. Ford Federal Direct Loan (Direct Loan) and Federal Family Education... DEPARTMENT OF EDUCATION Notice of Proposed Information Collection Requests; Federal Student Aid; William D. Ford Federal Direct Loan (Direct Loan) Program/ Federal Family Loan (FFEL) Program: Deferment...

  12. 22. Historic American Buildings Survey William F. Winter, Jr., Photographer ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    22. Historic American Buildings Survey William F. Winter, Jr., Photographer 1931 LATHE, THIRD FLOOR, NORTHEAST CORNER - Shaker Church Family Washhouse & Machine Shop, U.S. Route 20, Hancock, Berkshire County, MA

  13. Photographic copy of photograph (original print in possession of William ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    Photographic copy of photograph (original print in possession of William Langer Jewel Bearing Plant, Rolla, North Dakota). DRILLING - Turtle Mountain Ordnance Plant, 213 First Street Northwest, Rolla, Rolette County, ND

  14. Photographic copy of photograph (original print in possession of William ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    Photographic copy of photograph (original print in possession of William Langer Jewel Bearing Plant, Rolla, North Dakota). OLIVING - Turtle Mountain Ordnance Plant, 213 First Street Northwest, Rolla, Rolette County, ND

  15. 5. William Beardsley standing along canal section. Photographer James Dix ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    5. William Beardsley standing along canal section. Photographer James Dix Schuyler, 1903. Source: Schuyler report. - Waddell Dam, On Agua Fria River, 35 miles northwest of Phoenix, Phoenix, Maricopa County, AZ

  16. 8. William E. Barrett, Photographer, August 1975. LOG DOCK AND ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    8. William E. Barrett, Photographer, August 1975. LOG DOCK AND PARTIALLY DEMOLISHED JACKSLIP USED FOR HAULING LOGS UP TO SAWMILL. - Meadow River Lumber Company, Highway 60, Rainelle, Greenbrier County, WV

  17. 6. William E. Barrett, Photographer, 1975. GENERAL VIEW LOOKING EAST. ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    6. William E. Barrett, Photographer, 1975. GENERAL VIEW LOOKING EAST. SAWMILL ON RIGHT. SECONDARY WOODWORKING SHOPS ON LEFT PARTIALLY DEMOLISHED. - Meadow River Lumber Company, Highway 60, Rainelle, Greenbrier County, WV

  18. Bayesian stock assessment of Pacific herring in Prince William Sound, Alaska.

    PubMed

    Muradian, Melissa L; Branch, Trevor A; Moffitt, Steven D; Hulson, Peter-John F

    2017-01-01

    The Pacific herring (Clupea pallasii) population in Prince William Sound, Alaska crashed in 1993 and has yet to recover, affecting food web dynamics in the Sound and impacting Alaskan communities. To help researchers design and implement the most effective monitoring, management, and recovery programs, a Bayesian assessment of Prince William Sound herring was developed by reformulating the current model used by the Alaska Department of Fish and Game. The Bayesian model estimated pre-fishery spawning biomass of herring age-3 and older in 2013 to be a median of 19,410 mt (95% credibility interval 12,150-31,740 mt), with a 54% probability that biomass in 2013 was below the management limit used to regulate fisheries in Prince William Sound. The main advantages of the Bayesian model are that it can more objectively weight different datasets and provide estimates of uncertainty for model parameters and outputs, unlike the weighted sum-of-squares used in the original model. In addition, the revised model could be used to manage herring stocks with a decision rule that considers both stock status and the uncertainty in stock status.

  19. Bayesian stock assessment of Pacific herring in Prince William Sound, Alaska

    PubMed Central

    Moffitt, Steven D.; Hulson, Peter-John F.

    2017-01-01

    The Pacific herring (Clupea pallasii) population in Prince William Sound, Alaska crashed in 1993 and has yet to recover, affecting food web dynamics in the Sound and impacting Alaskan communities. To help researchers design and implement the most effective monitoring, management, and recovery programs, a Bayesian assessment of Prince William Sound herring was developed by reformulating the current model used by the Alaska Department of Fish and Game. The Bayesian model estimated pre-fishery spawning biomass of herring age-3 and older in 2013 to be a median of 19,410 mt (95% credibility interval 12,150–31,740 mt), with a 54% probability that biomass in 2013 was below the management limit used to regulate fisheries in Prince William Sound. The main advantages of the Bayesian model are that it can more objectively weight different datasets and provide estimates of uncertainty for model parameters and outputs, unlike the weighted sum-of-squares used in the original model. In addition, the revised model could be used to manage herring stocks with a decision rule that considers both stock status and the uncertainty in stock status. PMID:28222151

  20. Reevaluation of Monier-Williams method for determining sulfite in food

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Warner, C.R.; Daniels, D.H.; Joe, F.L. Jr.

    The Monier-Williams distillation procedure has a long history of successful use for determining sulfite in fruit products and wine; however, a systematic evaluation of its accuracy and precision with other food matrices has not been undertaken. The authors found that Monier-Williams distillation yielded >90% recovery of sulfite added to foods such as table grapes, hominy, dried mangoes, and lemon juice. Less than 85% recovery was obtained with broccoli, soda crackers, cheese-peanut butter crackers, mushrooms, and potato chips. These results may, in fact, accurately reflect the residual levels of sulfite if a portion of the sulfite undergoes irreversible reaction with somemore » food components. Analysis of commercial food products gave sulfite levels as 25 ppm in cream sherry.« less

  1. William J. Brennan Jr.: Judicial Architect of Affirmative Action.

    ERIC Educational Resources Information Center

    Eisler, Kim Isaac

    1997-01-01

    More than any other Supreme Court Justice, William Brennan worked to level the playing field for black Americans. As the recognized architect of affirmative action strategies for higher education, he left an indelible imprint on equal education in America. (SLD)

  2. Astronaut William Gregory prepares to exit his sleep quarters

    NASA Technical Reports Server (NTRS)

    1995-01-01

    Astronaut William G. Gregory, STS-67 pilot, ejects a cassette and prepares to bail out of his sleep quarters aboard the Earth orbiting Space Shuttle Endeavour. The astronaut was about to begin a shift of support to the red team.

  3. 8. Historic American Buildings Survey, William Heroy, Photographer November 25, ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    8. Historic American Buildings Survey, William Heroy, Photographer November 25, 1978 FIRST FLOOR, MAIN LOBBY LOOKING TOWARDS THE DRUG STORE. - O. Henry Hotel, North Elm & Bellemeade Streets, Greensboro, Guilford County, NC

  4. Photographic copy of photograph (original print in possession of William ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    Photographic copy of photograph (original print in possession of William Langer Jewel Bearing Plant, Rolla, North Dakota). MACHINE SHOP - Turtle Mountain Ordnance Plant, 213 First Street Northwest, Rolla, Rolette County, ND

  5. Photographic copy of photograph (original print in possession of William ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    Photographic copy of photograph (original print in possession of William Langer Jewel Bearing Plant, Rolla, North Dakota). FINAL INSPECTION - Turtle Mountain Ordnance Plant, 213 First Street Northwest, Rolla, Rolette County, ND

  6. 2. Historic American Buildings Survey William C. Everhart, Photographer October ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    2. Historic American Buildings Survey William C. Everhart, Photographer October 1958 VIEW FROM THE NORTHEAST (Residence of Caretaker at Left) - Sacred Heart Mission, Interstate 90 & Interchange 39, Cataldo, Shoshone County, ID

  7. 76 FR 13667 - Commercial Furniture Group, Inc., Formerly Known as Falcon Products, Inc., Shelby Williams, Howe...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-03-14

    ... Furniture Group, Inc., Formerly Known as Falcon Products, Inc., Shelby Williams, Howe and Thonet, Including...., Formerly Known as Falcon Products, Inc., Shelby Williams, Howe and Thonet, Chicago, IL; Amended Certification Regarding Eligibility To Apply for Worker Adjustment Assistance In accordance with Section 223 of...

  8. Inventing Orientation and Mobility Techniques and Teaching Methods: A Conversation with Russell Williams (Part 2)

    ERIC Educational Resources Information Center

    Welsh, Rachard L.

    2005-01-01

    This is the final part of the adaptation from my on-stage conversation with Russell Williams at the 1998 International Mobility conference in Atlanta, GA, which attempted to highlight Williams's contributions to the progression of orientation and mobility from the Army's immediate response to the service men and women who lost their sight during…

  9. A Teacher's Guide for William Shakespeare's "Henry V."

    ERIC Educational Resources Information Center

    WGBH-TV, Boston, MA.

    This teacher's guide for William Shakespeare's play "Henry V" is designed to accompany the Kenneth Branagh Masterpiece Theater film production of the play, and to help teachers use the film in a variety of ways. The guide includes pre-viewing background information, five teaching units, and a pullout poster for classroom display. The…

  10. A salute to William D. Novelli and AARP.

    PubMed

    2006-11-01

    William D. Novelli is the CEO of AARP, the membership organization which represents more than 40 million people over the age of 50. He has made AARP not only the largest, but the most respected association in Washington, D.C. He is known nationwide for his advocacy as well as his philanthropy.

  11. Report of Some Comets: The Discovery of Uranus and Comets by William, Caroline, and John Herschel

    NASA Astrophysics Data System (ADS)

    Pasachoff, Jay M.; Olson, R. J. M.

    2011-01-01

    We report on the discovery and drawings of comets by William, Caroline, and John Herschel. The first discovery, by William Herschel, in 1781 from Bath, published in the Philosophical Transactions of the Royal Society with the title "Report of a Comet," turned out to be Uranus, the first planet ever discovered, Mercury through Saturn having been known since antiquity. William's sister Caroline was given duties of sweeping the skies and turned out to be a discoverer of 8 comets in her own right, in addition to keeping William's notes. Caroline's comets were discovered from Slough between 1786 and 1797. In the process, we also discuss original documents from the archives of the Royal Society and of the Royal Astronomical Society. We conclude by showing comet drawings that we have recently attributed to John Herschel, including Halley's Comet from 1836, recently located in the Ransom Center of the University of Texas at Austin. Acknowledgments: Planetary astronomy at Williams College is supported in part by grant NNX08AO50G from NASA Planetary Astronomy. We thank Peter Hingley of the Royal Astronomical Society and Richard Oram of the Harry Ransom Center of The University of Texas at Austin for their assistance.

  12. Photographic copy of photograph (original print in possession of William ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    Photographic copy of photograph (original print in possession of William Langer Jewel Bearing Plant, Rolla, North Dakota). LARGE HOLE-OPENING - Turtle Mountain Ordnance Plant, 213 First Street Northwest, Rolla, Rolette County, ND

  13. 11. Historic American Buildings Survey William H. Knowles Collection Original: ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    11. Historic American Buildings Survey William H. Knowles Collection Original: About 1880 Re- photo: January 1940 VIEW FROM SOUTHEAST - Mission San Antonio de Padua, Hunter Liggett Military Reservation, Jolon, Monterey County, CA

  14. WE-G-213-02: The AAPM Award Eponyms: William D. Coolidge, Edith H. Quimby, and Marvin M.D. Williams - Who Were They and What Did They Do?

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Rothenberg, L.

    Roentgen and the Birth of Modern Medical Physics – Perry Sprawls Wilhelm Roentgen is well known for his discovery of x-radiation. What is less known and appreciated is his intensive research following the discovery to determine the characteristics of the “new kind of radiation” and demonstrate its great value for medical purposes. In this presentation we will imagine ourselves in Roentgen’s mind and follow his thinking, including questions and doubts, as he designs and conducts a series of innovative experiments that provided the foundation for the rapid growth of medical physics. Learning Objectives: Become familiar with the personal characteristics andmore » work of Prof. Roentgen that establishes him as an inspiring model for the medical physics profession. Observe the thought process and experiments that determined and demonstrated the comprehensive characteristics of x-radiation. The AAPM Award Eponyms: William D. Coolidge, Edith H. Quimby, and Marvin M.D. Williams - Who were they and what did they do? – Lawrence N. Rothenberg William David Coolidge (1873–1975) William Coolidge was born in Hudson, NY in 1873. He obtained his BS at the Massacusetts Institute of Technology in 1896. Coolidge then went to the University of Leipzig, Germany for graduate study with physicists Paul Drude and Gustave Wiedemann and received a Ph.D. in 1899. While in Germany he met Wilhelm Roentgen. Coolidge returned to the US to teach at MIT where he was associated with Arthur A. Noyes of the Chemistry Department, working on the electrical conductivity of aqueous solutions. Willis R. Whitney, under whom Coolidge had worked before going to Germany, became head of the newly formed General Electric Research Laboratory and he invited Coolidge to work with him. In 1905, Coolidge joined the staff of the GE laboratory and was associated with it for the remainder of his life. He developed ductile tungsten filaments to replace fragile carbon filaments as the material for electric

  15. 32. William E. Barrett, Photographer, 1973. DEADEND WELL WITH WOODEN ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    32. William E. Barrett, Photographer, 1973. DEAD-END WELL WITH WOODEN OIL TANK, PIPE ROCKER ARM. - West Oil Company Endless Wire Pumping Station, U.S. Route 50 (Volcano vicinity), Petroleum, Ritchie County, WV

  16. 24. INTERIOR VIEW, WILLIAM GRAY AT SIZING GUAGE ADJACENT TO ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    24. INTERIOR VIEW, WILLIAM GRAY AT SIZING GUAGE ADJACENT TO BRADLEY HAMMER; NOTE THIS IS THE SAME TOOL AS BEING FORGED ABOVE - Warwood Tool Company, Foot of Nineteenth Street, Wheeling, Ohio County, WV

  17. Orientation Perception in Williams Syndrome: Discrimination and Integration

    ERIC Educational Resources Information Center

    Palomares, Melanie; Landau, Barbara; Egeth, Howard

    2009-01-01

    Williams Syndrome (WS) is a rare neurodevelopmental disorder, which stems from a genetic deletion on chromosome 7 and causes a profound weakness in visuospatial cognition. Our current study explores how orientation perception may contribute to the visuospatial deficits in WS. In Experiment 1, we found that WS individuals and normal 3-4 year olds…

  18. Williams with TVIS hardware in Zvezda Service module

    NASA Image and Video Library

    2007-02-27

    ISS014-E-15349 (27 Feb. 2007) --- Assisted by the weightlessness of space, astronaut Sunita L. Williams, Expedition 14 flight engineer, hoists the Treadmill Vibration Isolation System (TVIS). She took a moment to pose for a photo during routine in-flight maintenance (IFM) on TVIS in the Zvezda Service Module of the International Space Station.

  19. Astronaut Sunita L. Williams Submerges Into Waters of the Neutral Buoyancy Laboratory (NBL)

    NASA Technical Reports Server (NTRS)

    2006-01-01

    Astronauts Sunita L. Williams, Expedition 14 flight engineer, and Robert L. Curbeam (partially obscured), STS-116 mission specialist, are about to be submerged in the waters of the Neutral Buoyancy Laboratory (NBL) near Johnson Space Center. Williams and Curbeam are attired in training versions of the Extravehicular Mobility Unit (EMU) space suit. SCUBA-equipped divers are in the water to assist the crew members in their rehearsal intended to help prepare them for work on the exterior of the International Space Station (ISS).

  20. Nuclear and mitochondrial markers reveal evidence for genetically segregated cryptic speciation in giant Pacific octopuses from Prince William Sound, Alaska

    USGS Publications Warehouse

    Toussaint, Rebecca K.; Scheel, David; Sage, G.K.; Talbot, S.L.

    2012-01-01

    Multiple species of large octopus are known from the north Pacific waters around Japan, however only one large species is known in the Gulf of Alaska (the giant Pacific octopus, Enteroctopus dofleini). Current taxonomy of E. dofleini is based on geographic and morphological characteristics, although with advances in genetic technology that is changing. Here, we used two mitochondrial genes (cytochrome b and cytochrome oxidase I), three nuclear genes (rhodopsin, octopine dehydrogenase, and paired-box 6), and 18 microsatellite loci for phylogeographic and phylogenetic analyses of octopuses collected from across southcentral and the eastern Aleutian Islands (Dutch Harbor), Alaska. Our results suggest the presence of a cryptic Enteroctopus species that is allied to, but distinguished from E. dofleini in Prince William Sound, Alaska. Existence of an undescribed and previously unrecognized taxon raises important questions about the taxonomy of octopus in southcentral Alaska waters.

  1. Hydrologic conditions in the Bill Williams River National Wildlife Refuge and Planet Valley, Arizona, 2000

    USGS Publications Warehouse

    Wilson, Richard P.; Owen-Joyce, Sandra J.

    2002-01-01

    During a period of sustained base-flow conditions in the Bill Williams River below Alamo Dam in west central Arizona from March to July 2000, the channel of the river through Planet Valley was dry, and the water table sloped almost due west parallel to the main slope of the flood plain. Water from the river infiltrated into the channel bottom at the head of Planet Valley, moved downgradient in the subsurface, and reappeared in the channel about 0.3 mile downstream from the east boundary of the Bill Williams River National Wildlife Refuge. A river aquifer in hydraulic connection with the Bill Williams River was mapped from a point 6.3 miles upstream from Highway 95 to the upstream end of Planet Valley. Formations that make up the river aquifer in Planet Valley are younger alluvium, older alluviums, and fanglomerate. Total thickness of the river aquifer probably is less than 200 feet in the bedrock canyons to as much as 1,035 feet in Planet Valley. The purpose of this study was to investigate the current hydrologic conditions along the Bill Williams River, which included an inventory of wells within the river aquifer of the Colorado River and in Planet Valley, and to determine the configuration of the water table. A map shows the elevation and configuration of the water table from the east end of Planet Valley to the confluence of the Bill Williams River with Lake Havasu.

  2. William James, Nitrous Oxide, and the Anaesthetic Revelation.

    PubMed

    Moon, Jane S; Kuza, Catherine M; Desai, Manisha S

    2018-01-01

    William James greatly influenced the fields of psychology, philosophy, and religion during the late 19 th and early 20 th centuries. This was the era of Modernism, a time when many writers rejected the certainty of Enlightenment ideals. Positivism, which rose to prominence in the early 19th century, had emphasized physical phenomena, empirical evidence, and the scientific method. Darwin's On the Origin of Species (1859), with its theory of natural selection, provided an explanation for the evolution of species apart from a divine Creator. Within this context, William James served as a "mediator between scientific agnosticism and the religious view of the world." James' own experience inhaling nitrous oxide played an important role in shaping his views. For James, the use of nitrous oxide served a key role in elucidating some of his most central ideas: 1) the value of religion, and the emphasis on mysticism and revelation (as opposed to theology and doctrine) as religion's foundation; 2) the universe as pluralistic (as opposed to absolutist, constant, eternal), driven by chance, experience, and change. Copyright © 2017 Elsevier Inc. All rights reserved.

  3. Health and Social Outcomes in Adults with Williams Syndrome: Findings from Cross-Sectional and Longitudinal Cohorts

    ERIC Educational Resources Information Center

    Elison, Sarah; Stinton, Chris; Howlin, Patricia

    2010-01-01

    Previous studies have investigated trajectories of cognitive, language and adaptive functioning in Williams syndrome (WS) but little is known about how other aspects of the Williams syndrome behavioural phenotype change across the life-span. Therefore, the present study examined age associated changes in a number of different domains of…

  4. 8. Historic American Buildings Survey, William Heroy, Photographer February, 1979 ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    8. Historic American Buildings Survey, William Heroy, Photographer February, 1979 FIRST FLOOR OF GREENSBORO MOTOR COMPANY, SHOWROOM INTERIOR. - Buick Motor Company & Greensboro Motor Company Dealerships, 309 & 315 North Elm Street, Greensboro, Guilford County, NC

  5. 14. Photocopy of photograph (from William H. Knowles Collection, c. ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    14. Photocopy of photograph (from William H. Knowles Collection, c. 1880) EXTERIOR, SOUTH FRONT AND WEST SIDE OF MISSION, C. 1880 - Mission San Francisco Solano de Sonoma, First & Spain Streets, Sonoma, Sonoma County, CA

  6. 2. Historic American Buildings Survey, William F. Winter, Jr., Photographer ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    2. Historic American Buildings Survey, William F. Winter, Jr., Photographer 1920's, EXTRACTING ROOM, Gift of New York State Department of Education. - Shaker Centre Family Medicine Factory, Shaker Road, New Lebanon, Columbia County, NY

  7. The work experience of a patient affected by Williams Syndrome: a pilot project at the Bambino Gesù Children's Hospital.

    PubMed

    De Lorenzo, Francesca; Macchiaiolo, Marina; Carlevaris, Carla Maria; Bartuli, Andrea

    2017-05-31

    A new approach has been designed at the Bambino Gesù Children's Hospital in Rome aimed at increasing empowerment in Williams Syndrome individuals through tutor-assisted work activities. Williams Syndrome is characterized by a combination of distinguishing physical traits, congenital anomalies, intellectual disabilities, and a specific developmental profile.This manuscript describes the case of a Williams Syndrome patient.There are only few papers in the scientific literature describing interventions targeting improvement in the quality of life of adult Williams Syndrome individuals. Therefore, this experience may prove useful to several patients, their families, and the experts helping them.We described an example of intervention aimed at guiding and facilitating a Williams Syndrome patient within a work environment, taking into consideration the peaks and valleys of these individuals' specific abilities.Based on our results, we also stressed the need to promote a set of projects and initiatives aimed at enhancing as much as possible self-sufficiency and psycho-affective balance in Williams Syndrome individuals, in order to protect their dignity and self-esteem.

  8. A Conversation with William A. Fowler Part II

    NASA Astrophysics Data System (ADS)

    Greenberg, John

    2005-06-01

    Physicist William A.Fowler initiated an experimental program in nuclear astrophysics after World War II. He recalls here the Steady State versus Big Bang controversy and his celebrated collaboration with Fred Hoyle and Geoffrey and Margaret Burbidge on nucleosynthesis in stars. He also comments on the shift away from nuclear physics in universities to large accelerators and national laboratories.

  9. Advocating for Inclusion of Children with Williams Syndrome

    ERIC Educational Resources Information Center

    Self, Michelle A.

    2010-01-01

    The purpose of this study was to describe and explore the experience of inclusion of students with Williams syndrome, a rare genetic condition of a microdeletion on chromosome 7 which has medical, behavior, and cognitive issues. The study was conducted by gaining an understanding from the parents' point of view. The study was twofold. First, the…

  10. Discourse Analysis of Tennessee Williams' "The Glass Menagerie"

    ERIC Educational Resources Information Center

    Nalliveettil, George Mathew; Gadallah, Mahmoud Sobhi Mohamed

    2016-01-01

    "The Glass Menagerie" is one of the Tennessee Williams' most famous plays which won the New York Drama Critics' Circle award. It elevated him to be one of the greatest playwrights of his generation. As a playwright, he is skilful to make the readers conscious of the unconscious habits and attitudes in everyday life. In "The Glass…

  11. Williams works with LOCAD-PTS in Destiny lab

    NASA Image and Video Library

    2007-04-01

    ISS014-E-18822 (31 March 2007) --- Astronaut Sunita L. Williams, Expedition 14 flight engineer, works with the Lab-on-a-Chip Application Development-Portable Test System (LOCAD-PTS) experiment in the Destiny laboratory of the International Space Station. LOCAD-PTS is a handheld device for rapid detection of biological and chemical substances onboard the station.

  12. Williams works with LOCAD-PTS in Destiny lab

    NASA Image and Video Library

    2007-04-01

    ISS014-E-18818 (31 March 2007) --- Astronaut Sunita L. Williams, Expedition 14 flight engineer, works with the Lab-on-a-Chip Application Development-Portable Test System (LOCAD-PTS) experiment in the Destiny laboratory of the International Space Station. LOCAD-PTS is a handheld device for rapid detection of biological and chemical substances onboard the station.

  13. Williams works with LOCAD-PTS in Destiny lab

    NASA Image and Video Library

    2007-04-01

    ISS014-E-18811 (31 March 2007) --- Astronaut Sunita L. Williams, Expedition 14 flight engineer, works with the Lab-on-a-Chip Application Development-Portable Test System (LOCAD-PTS) experiment in the Destiny laboratory of the International Space Station. LOCAD-PTS is a handheld device for rapid detection of biological and chemical substances onboard the station.

  14. Mapping the Milky Way: William Herschel's Star Gages

    ERIC Educational Resources Information Center

    Timberlake, Todd

    2013-01-01

    William Herschel (Fig. 1) is rightfully known as one of the greatest astronomers of all time. Born in Hanover (in modern Germany) in 1738, Herschel immigrated to England in 1757 and began a successful career as a professional musician. Later in life Herschel developed a strong interest in astronomy. He began making his own reflecting telescopes in…

  15. [William Harvey revisited ].

    PubMed

    Steinke, Hubert

    2015-07-01

    William Harvey's discovery of the circulation of the blood is often described as a product of the Scientific Revolution of the Seventeenth Century. Modern research has, however, shown thatHarvey followed the Aristotelian research tradition and thus tried to reveal the purpose of the organs through examination of various animals. His publication of 1628 has to be read as an argument of natural philosophy, or, more precisely, as a series of linked observations, experiments and philosophical reasonings from which the existence of circulation has to be deduced as a logical consequence. Harvey did not consider experiments as superior to philosophical reasoning nor intended he to create a new system of medicine. He believed in the vitality of the heart and the blood and rejected Francis Bacon's empirism and the mechanistic rationalism of Descartes. Harvey's contribution and originality lied less in his single observations and experiments but in the manner how he linked them with critical reasoning and how he accepted, presented and defended the ensuing radical findings.

  16. 3. Historic American Buildings Survey, William F. Winter, Jr., Photographer ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    3. Historic American Buildings Survey, William F. Winter, Jr., Photographer Summer 1931, EAST (FRONT) SIDE, Gift of New York State Department of Education. - Shaker North Family, Dwelling House, Shaker Road, New Lebanon, Columbia County, NY

  17. Photographic copy of photograph (original print in possession of William ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    Photographic copy of photograph (original print in possession of William Langer Jewel Bearing Plant, Rolla, North Dakota). PASTING JEWEL BLANKS, PREPARATION FOR DRILLING. - Turtle Mountain Ordnance Plant, 213 First Street Northwest, Rolla, Rolette County, ND

  18. 1. Historic American Buildings Survey, William F. Winter, Jr., Photographer ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    1. Historic American Buildings Survey, William F. Winter, Jr., Photographer 1920's, VIEW FROM SOUTHEAST, Gift of New York State Department of Education. - Shaker Church Family Seed House, Shaker Road, New Lebanon, Columbia County, NY

  19. From Father to Son: Generative Care and Gradual Conversion in William James's Writing of "The Varieties"

    ERIC Educational Resources Information Center

    Bridgers, Lynn; Snarey, John R.

    2003-01-01

    Using a historical and biographical, then developmental, approach, this article examines William James's spiritual family history by reviewing key events in the life of his father, Henry James, Sr. It pays particular attention to Henry Sr's tumultuous relationship with his own father, William James of Albany, and Henry Sr's subsequent conversion…

  20. Sandstone petrographic evidence and the Chugach-Prince William terrane boundary in southern Alaska

    USGS Publications Warehouse

    Dumoulin, Julie A.

    1988-01-01

    The contact between the Upper Cretaceous Valdez Group and the Paleocene and Eocene Orca Group has been inferred to be the boundary between the Chugach and the Prince William tectonostratigraphic terranes. Sandstone petrographic data from the Prince William Sound area show no compositional discontinuity across this contact. These data are best explained by considering the Valdez and Orca Groups to be part of a single terrane - a thick flysch sequence derived primarily from a progressively unroofing magmatic arc with increasing input from subduction-complex sources through time.

  1. William Penn and the peace of Europe.

    PubMed

    Russell, W M S

    2004-01-01

    The Quaker William Penn proposed a European Union to ensure peace in the continent in 1693. Penn was unusual among Quakers in being of the landed upper classes. When converted, he became a leader of the Quakers and other Dissenters. He had the two related ideals of peace and religious toleration, and dreamed of realizing both ideals in the New World. A practical idealist, he took advantage of four factors: friends at Court made through his social position; King Charles II's gratitude for services rendered by his father, Admiral Sir William Penn; the King's desire to conciliate the City merchants, who were ready to invest in Penn's scheme; and above all the King's concern to get North America settled by British colonists. Penn received a charter to found Pennsylvania in 1681. In England he worked hard, especially in collaboration with James II, for toleration for the cruelly persecuted Quakers and other Dissenters. In Pennsylvania he was able to establish complete toleration and his fair and friendly treatment gave the colony 70 years of peaceful co-existence with the Indians. In his essay on the peace of Europe, he virtually invented collective security and with amazing foresight planned in detail something very like the present European Union.

  2. Two-Dimensional Ffowcs Williams/Hawkings Equation Solver

    NASA Technical Reports Server (NTRS)

    Lockard, David P.

    2005-01-01

    FWH2D is a Fortran 90 computer program that solves a two-dimensional (2D) version of the equation, derived by J. E. Ffowcs Williams and D. L. Hawkings, for sound generated by turbulent flow. FWH2D was developed especially for estimating noise generated by airflows around such approximately 2D airframe components as slats. The user provides input data on fluctuations of pressure, density, and velocity on some surface. These data are combined with information about the geometry of the surface to calculate histories of thickness and loading terms. These histories are fast-Fourier-transformed into the frequency domain. For each frequency of interest and each observer position specified by the user, kernel functions are integrated over the surface by use of the trapezoidal rule to calculate a pressure signal. The resulting frequency-domain signals are inverse-fast-Fourier-transformed back into the time domain. The output of the code consists of the time- and frequency-domain representations of the pressure signals at the observer positions. Because of its approximate nature, FWH2D overpredicts the noise from a finite-length (3D) component. The advantage of FWH2D is that it requires a fraction of the computation time of a 3D Ffowcs Williams/Hawkings solver.

  3. 1. Historic American Buildings Survey, William F. Winter, Jr., Photographer ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    1. Historic American Buildings Survey, William F. Winter, Jr., Photographer Spring 1931, GENERAL VIEW - SHOPS, Gift of New York State Department of Education. - Shaker Upper Canaan Family, Shop Buildings, Shaker Road, New Lebanon, Columbia County, NY

  4. 11. William E. Barrett, Photographer, August 1975. INTERIOR OF SAWMILL ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    11. William E. Barrett, Photographer, August 1975. INTERIOR OF SAWMILL SHOWING STEAM CYLINDER AND PISTON ROD OF LEFT-HAND MILL (LEFT) AND HOUSING OF CENTER BANDSAW. - Meadow River Lumber Company, Highway 60, Rainelle, Greenbrier County, WV

  5. 16. William E. Barrett, Photographer, August 1975. CENTER MILL (RIGHTHANDED) ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    16. William E. Barrett, Photographer, August 1975. CENTER MILL (RIGHT-HANDED) WITH CARRIAGE TRACKS AND BANDSAW HOUSING INTACT. LOG CLAMPS ON THIS CARRIAGE WERE PNEUMATICALLY OPERATED. - Meadow River Lumber Company, Highway 60, Rainelle, Greenbrier County, WV

  6. 27. Ladle car (William B. Pollock Co., Youngstown, OH, no ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    27. Ladle car (William B. Pollock Co., Youngstown, OH, no date) for transporting molten iron from furnace to pig machine - Sloss-Sheffield Steel & Iron, First Avenue North Viaduct at Thirty-second Street, Birmingham, Jefferson County, AL

  7. 3. Historic American Buildings Survey, William F. Winter, Jr., Photographer ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    3. Historic American Buildings Survey, William F. Winter, Jr., Photographer August 1931, BOTTLING AND PACKING ROOM, Gift of New York State Department of Education. - Shaker Centre Family Medicine Factory, Shaker Road, New Lebanon, Columbia County, NY

  8. 1. Historic American Buildings Survey, William F. Winter, Jr., Photographer ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    1. Historic American Buildings Survey, William F. Winter, Jr., Photographer June 1931, NORTH AND SOUTH ELEVATIONS, Gift of New York State Department of Education. - Shaker Centre Family Medicine Factory, Shaker Road, New Lebanon, Columbia County, NY

  9. Characterisation of Sleep Problems in Children with Williams Syndrome

    ERIC Educational Resources Information Center

    Annaz, Dagmara; Hill, Catherine M.; Ashworth, Anna; Holley, Simone; Karmiloff-Smith, Annette

    2011-01-01

    Sleep is critical to optimal daytime functioning, learning and general health. In children with established developmental disorders sleep difficulties may compound existing learning difficulties. The purpose of the present study was to evaluate the prevalence and syndrome specificity of sleep problems in Williams syndrome (WS), a…

  10. Astronaut William Gregory works with pharmaceutical experiments on middeck

    NASA Technical Reports Server (NTRS)

    1995-01-01

    Astronaut William G. Gregory, STS-67 pilot, works with a pharmaceutical experiment on the middeck of the Earth-orbiting Space Shuttle Endeavour. Commercial Materials Dispersion Apparatus Instruments Technology Associates Experiments (CMIX-03) includes not only pharmaceutical but also biotechnology, cell biology, fluids and crystal growth investigations.

  11. 78 FR 63464 - William D. Ford Federal Direct Loan Program Repayment Plan Selection Form; Extension of Public...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-10-24

    ... DEPARTMENT OF EDUCATION William D. Ford Federal Direct Loan Program Repayment Plan Selection Form; Extension of Public Comment Period; Correction AGENCY: Department of Education. ACTION: Correction notice... entitled, ``William D. Ford Federal Direct Loan Program Repayment Plan Selection Form''. ED is extending...

  12. 1. Historic American Buildings Survey, William F. Winter, Jr., Photographer ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    1. Historic American Buildings Survey, William F. Winter, Jr., Photographer August 1931, EXTERIOR VIEW OF KILN, Gift of New York State Department of Education. - Shaker Church Family Apple Drying Kiln, Shaker Road, New Lebanon, Columbia County, NY

  13. 3. Historic American Buildings Survey, William F. Winter, Jr., Photographer, ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    3. Historic American Buildings Survey, William F. Winter, Jr., Photographer, September 1926, VIEW FROM NORTHWEST, Gift of New York State Department of Education. - Shaker Church Family, Herb House, Watervliet Shaker Road, Colonie Township, Watervliet, Albany County, NY

  14. 3. Historic American Buildings Survey, William F. Winter, Jr., Photographer, ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    3. Historic American Buildings Survey, William F. Winter, Jr., Photographer, April 1926, VIEW FROM SOUTHWEST, Gift of New York State Department of Education. - Shaker Church Family Brethren's Workshop, Watervliet Shaker Road, Colonie Township, Watervliet, Albany County, NY

  15. 4. Historic American Buildings Survey, William F. Winter, Jr., Photographer, ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    4. Historic American Buildings Survey, William F. Winter, Jr., Photographer, April 1925, VIEW FROM SOUTHWEST, Gift of New York State Department of Education. - Shaker Church Family Brethren's Workshop, Watervliet Shaker Road, Colonie Township, Watervliet, Albany County, NY

  16. 2. Historic American Buildings Survey, William F. Winter, Jr., Photographer ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    2. Historic American Buildings Survey, William F. Winter, Jr., Photographer 1920's, EAST (FRONT) AND NORTH SIDES, Gift of New York State Department of Education. - Shaker North Family, Dwelling House, Shaker Road, New Lebanon, Columbia County, NY

  17. 4. Historic American Buildings Survey, William F. Winter, Jr., Photographer ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    4. Historic American Buildings Survey, William F. Winter, Jr., Photographer Summer 1930, WEST (REAR) AND NORTH SIDES, Gift of New York State Department of Education. - Shaker North Family, Dwelling House, Shaker Road, New Lebanon, Columbia County, NY

  18. 1. Historic American Buildings Survey, William F. Winter, Jr., Photographer, ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    1. Historic American Buildings Survey, William F. Winter, Jr., Photographer, 1920's, VIEW FROM NORTHWEST, Gift of New York State Department of Education. - Shaker Church Family Brethren's Workshop, Watervliet Shaker Road, Colonie Township, Watervliet, Albany County, NY

  19. 1. Historic American Buildings Survey, William F. Winter, Jr., Photographer, ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    1. Historic American Buildings Survey, William F. Winter, Jr., Photographer, September 1926, VIEW FROM SOUTHWEST, Gift of New York State Department of Education. - Shaker Church Family Seed House, Watervliet Shaker Road, Colonie Township, Watervliet, Albany County, NY

  20. 74. ARAII. Dr. William Zinn of combustion engineering company and ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    74. ARA-II. Dr. William Zinn of combustion engineering company and others at controls of SL-1. August 8, 1959. Ineel photo no. 59-4109. - Idaho National Engineering Laboratory, Army Reactors Experimental Area, Scoville, Butte County, ID

  1. 13. William E. Barrett, Photographer, August 1975. INTERIOR OF SAWMILL ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    13. William E. Barrett, Photographer, August 1975. INTERIOR OF SAWMILL LOOKING EAST. LOGS FOR CENTER MILL ROLLED RIGHT TO CONVEYOR WHICH CARRIED THEM TO SECOND SLOPING DECK. - Meadow River Lumber Company, Highway 60, Rainelle, Greenbrier County, WV

  2. William Herschel, the First Observational Cosmologist

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Lemonick, Michael

    In the late 1700s, a composer, orchestra director and soloist named William Herschel became fascinated with astronomy, and, having built his own reflecting telescope, went out in his garden in Bath, England, one night and discovered Uranus—the first planet in human history ever found by an individual. The feat earned him a lifetime pension from King George III. But Herschel considered the discovery to be relatively unimportant in comparison to his real work: understanding the composition, structure and evolution of the universe. In pursuing that work, he became the first observational cosmologist.

  3. Speech fluency profile in Williams-Beuren syndrome: a preliminary study.

    PubMed

    Rossi, Natalia Freitas; Souza, Deise Helena de; Moretti-Ferreira, Danilo; Giacheti, Célia Maria

    2009-01-01

    the speech fluency pattern attributed to individuals with Williams-Beuren syndrome (WBS) is supported by the effectiveness of the phonological loop. Some studies have reported the occurrence of speech disruptions caused by lexical and semantic deficits. However, the type and frequency of such speech disruptions has not been well elucidated. to determine the speech fluency profile of individuals with WBS and to compare the speech performance of these individuals to a control group matched by gender and mental age. Twelve subjects with Williams-Beuren syndrome, chronologically aged between 6.6 and 23.6 years and mental age ranging from 4.8 to 14.3 years, were evaluated. They were compared with another group consisting of 12 subjects with similar mental age and with no speech or learning difficulties. Speech fluency parameters were assessed according to the ABFW Language Test: type and frequency of speech disruptions and speech rate. The obtained results were compared between the groups. In comparison with individuals of similar mental age and typical speech and language development, the group with Williams-Beuren syndrome showed a greater percentage of speech discontinuity, and an increased frequency of common hesitations and word repetition. The speech fluency profile presented by individuals with WBS in this study suggests that the presence of disfluencies can be caused by deficits in the lexical, semantic, and syntactic processing of verbal information. The authors stress that further systematic investigations on the subject are warranted.

  4. Comprehension of Sarcasm, Metaphor and Simile in Williams Syndrome

    ERIC Educational Resources Information Center

    Godbee, Kali; Porter, Melanie

    2013-01-01

    Background: Although people with Williams syndrome (WS) are often characterized as friendly and sociable with relatively good general language abilities, there is emerging evidence of pragmatic difficulties and trouble comprehending aspects of non-literal language. Aims: The main aim was to investigate the comprehension of sarcasm, metaphor and…

  5. Gender Attribution and Gender Agreement in French Williams Syndrome

    ERIC Educational Resources Information Center

    Boloh, Yves; Ibernon, Laure; Royer, Stephanie; Escudier, Frederique; Danillon, Aurelia

    2009-01-01

    Previous studies on grammatical gender in French individuals with Williams syndrome (WS) have led to conflicting findings and interpretations regarding keys abilities--gender attribution and gender agreement. New production data from a larger SW sample (N = 24) showed that gender attribution scores in SW participants exactly mirrored those of…

  6. Visually Guided Step Descent in Children with Williams Syndrome

    ERIC Educational Resources Information Center

    Cowie, Dorothy; Braddick, Oliver; Atkinson, Janette

    2012-01-01

    Individuals with Williams syndrome (WS) have impairments in visuospatial tasks and in manual visuomotor control, consistent with parietal and cerebellar abnormalities. Here we examined whether individuals with WS also have difficulties in visually controlling whole-body movements. We investigated visual control of stepping down at a change of…

  7. Object Recognition in Williams Syndrome: Uneven Ventral Stream Activation

    ERIC Educational Resources Information Center

    O'Hearn, Kirsten; Roth, Jennifer K.; Courtney, Susan M.; Luna, Beatriz; Street, Whitney; Terwillinger, Robert; Landau, Barbara

    2011-01-01

    Williams syndrome (WS) is a genetic disorder associated with severe visuospatial deficits, relatively strong language skills, heightened social interest, and increased attention to faces. On the basis of the visuospatial deficits, this disorder has been characterized primarily as a deficit of the dorsal stream, the occipitoparietal brain regions…

  8. Strategies and Biases in Location Memory in Williams Syndrome

    ERIC Educational Resources Information Center

    Farran, Emily K.

    2008-01-01

    Individuals with Williams syndrome (WS) demonstrate impaired visuo-spatial abilities in comparison to their level of verbal ability. In particular, visuo-spatial construction is an area of relative weakness. It has been hypothesised that poor or atypical location coding abilities contribute strongly to the impaired abilities observed on…

  9. William Cruikshank (1745-1800), anatomist and surgeon, and his illustrious patient, Samuel Johnson.

    PubMed

    McDonald, Stuart W

    2015-10-01

    William Cumberland Cruikshank (1745-1800) was a Scot who from 1771 until his death taught anatomy at the famous school of anatomy in Great Windmill Street, London, founded by William Hunter (1718-1783). Arguably, his most famous patient was Samuel Johnson, the celebrated 18th Century man of letters and author of the first English dictionary. This article, largely drawn from Johnson's correspondence, documents the medical condition that caused Johnson to consult Cruikshank and some of the social links between Johnson, Hunter, and Cruikshank. © 2015 Wiley Periodicals, Inc.

  10. 1. Historic American Buildings Survey, William F. Winter, Jr., Photographer ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    1. Historic American Buildings Survey, William F. Winter, Jr., Photographer Summer 1931, EAST SIDE, Gift of New York State Department of Education. - Shaker North Family Barn, State Route 22 & U.S. Route 20, New Lebanon, Columbia County, NY

  11. 3. Historic American Buildings Survey, William F. Winter, Jr., Photographer ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    3. Historic American Buildings Survey, William F. Winter, Jr., Photographer August 1931, SOUTH SIDES, Gift of New York State Department of Education. - Shaker North Family Barn, State Route 22 & U.S. Route 20, New Lebanon, Columbia County, NY

  12. 4. Historic American Buildings Survey, William F. Winter, Jr., Photographer ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    4. Historic American Buildings Survey, William F. Winter, Jr., Photographer Summer 1930, SOUTH WINGS, Gift of New York State Department of Education. - Shaker North Family Barn, State Route 22 & U.S. Route 20, New Lebanon, Columbia County, NY

  13. William Francis Rienhoff, Jr., MD - Halsted's Last Resident.

    PubMed

    Heitmiller, Richard F

    2017-12-01

    : William Francis Rienhoff Jr. was a skilled and innovative surgeon whose career spanned over 4 decades of patient care, clinical investigative research, and surgical education. He was an unforgettable character for those who knew him. Colleagues, coworkers, and friends developed strong and divergent opinions of him. His professional life coincided with the early development of general and thoracic surgery to which he contributed.

  14. Anaesthesia-related haemodynamic complications in Williams syndrome patients: a review of one institution's experience.

    PubMed

    Olsen, M; Fahy, C J; Costi, D A; Kelly, A J; Burgoyne, L L

    2014-09-01

    Williams syndrome is a genetic disorder associated with cardiac pathology, including supravalvular aortic stenosis and coronary artery stenosis. Sudden cardiac death has been reported in the perioperative period and attributed to cardiovascular pathology. In this retrospective audit, case note and anaesthetic records were reviewed for all confirmed Williams syndrome patients who had received an anaesthetic in our institution between July 1974 and November 2009. There were a total of 108 anaesthetics administered in 29 patients. Twelve of the anaesthetics (11.1%) were associated with cardiac complications including cardiac arrest in two cases (1.85%). Of the two cardiac arrests, one patient died within the first 24 hours postanaesthetic and the other patient survived, giving an overall mortality of 0.9% (3.4%). We conclude that Williams syndrome confers a significant anaesthetic risk, which should be recognised and considered by clinicians planning procedures requiring general anaesthesia.

  15. 75 FR 28822 - Duke Energy Carolina, LLC; William States Lee III Combined License Application; Notice of Intent...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-05-24

    ..., LLC; William States Lee III Combined License Application; Notice of Intent To Conduct a Supplemental... an application for combined licenses (COL) for its William States Lee III Nuclear Station (Lee) site.../new-licensing/col/lee.html . In addition, the Cherokee County Public Library, 300 E. Rutledge Avenue...

  16. 4. Historic American Buildings Survey, William F. Winter, Jr., Photographer, ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    4. Historic American Buildings Survey, William F. Winter, Jr., Photographer, April 1925, INTERIOR VIEW WITH HERB PRESS, Gift of New York State Department of Education. - Shaker Church Family, Herb House, Watervliet Shaker Road, Colonie Township, Watervliet, Albany County, NY

  17. An Investigation of Voice Quality in Individuals with Inherited Elastin Gene Abnormalities

    ERIC Educational Resources Information Center

    Watts, Christopher R.; Awan, Shaheen N.; Marler, Jeffrey A.

    2008-01-01

    The human elastin gene (ELN) is responsible for the generation of elastic fibres in the extracellular matrix of connective tissue throughout the body, including the vocal folds. Individuals with Supravalvular aortic stenosis (SVAS) and Williams syndrome (WS) lack one normal ELN allele due to heterozygous ELN abnormalities, resulting in a…

  18. 50 CFR Figure 4 to Subpart E of... - Prince William Sound Rural and Non-Rural Areas

    Code of Federal Regulations, 2014 CFR

    2014-10-01

    ... 50 Wildlife and Fisheries 11 2014-10-01 2014-10-01 false Prince William Sound Rural and Non-Rural Areas 4 Figure 4 to Subpart E of Part 300 Wildlife and Fisheries INTERNATIONAL FISHING AND RELATED... to Subpart E of Part 300—Prince William Sound Rural and Non-Rural Areas ER04NO09.010 [74 FR 57110...

  19. 50 CFR Figure 4 to Subpart E of... - Prince William Sound Rural and Non-Rural Areas

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ... 50 Wildlife and Fisheries 11 2013-10-01 2013-10-01 false Prince William Sound Rural and Non-Rural Areas 4 Figure 4 to Subpart E of Part 300 Wildlife and Fisheries INTERNATIONAL FISHING AND RELATED... to Subpart E of Part 300—Prince William Sound Rural and Non-Rural Areas ER04NO09.010 [74 FR 57110...

  20. 50 CFR Figure 4 to Subpart E of... - Prince William Sound Rural and Non-Rural Areas

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ... 50 Wildlife and Fisheries 11 2012-10-01 2012-10-01 false Prince William Sound Rural and Non-Rural Areas 4 Figure 4 to Subpart E of Part 300 Wildlife and Fisheries INTERNATIONAL FISHING AND RELATED... to Subpart E of Part 300—Prince William Sound Rural and Non-Rural Areas ER04NO09.010 [74 FR 57110...

  1. 50 CFR Figure 4 to Subpart E of... - Prince William Sound Rural and Non-Rural Areas

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 50 Wildlife and Fisheries 7 2010-10-01 2010-10-01 false Prince William Sound Rural and Non-Rural Areas 4 Figure 4 to Subpart E of Part 300 Wildlife and Fisheries INTERNATIONAL FISHING AND RELATED... to Subpart E of Part 300—Prince William Sound Rural and Non-Rural Areas ER04NO09.010 [74 FR 57110...

  2. 50 CFR Figure 4 to Subpart E of... - Prince William Sound Rural and Non-Rural Areas

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 50 Wildlife and Fisheries 9 2011-10-01 2011-10-01 false Prince William Sound Rural and Non-Rural Areas 4 Figure 4 to Subpart E of Part 300 Wildlife and Fisheries INTERNATIONAL FISHING AND RELATED... to Subpart E of Part 300—Prince William Sound Rural and Non-Rural Areas ER04NO09.010 [74 FR 57110...

  3. Sequence-stratigraphic controls on sandstone diagenesis: An example from the Williams Fork formation, Piceance Basin, Colorado

    NASA Astrophysics Data System (ADS)

    Aboktef, Adel

    This study documents the distribution of diagenetic alterations in Williams Fork fluvial sandstones, assess sequence stratigraphic controls on diagenetic features, and addresses diagenetic impacts on porosity. Petrographic point counts of 220 thin sections from six wells forms the database. The near absence of potassium feldspar and volcanic rock fragments in the lower Williams Fork interval and increasing plagioclase content upward represent changes in sediment provenance rather than stratigraphic variability in diagenesis. The lower Williams Fork sands are from sedimentary sources whereas middle and upper Williams Fork sands include input from magmatic arcs and basement uplifts. Compaction, early and late cementation, dissolution, and replacement by calcite or clay minerals combined to alter Williams Fork sandstones. Infiltration of clays occurred prior to any burial. Chlorite, quartz, non-ferroan calcite, compaction and dissolution features, and kaolinite formed during eo-diagenesis at <70°C. More quartz, compaction and dissolution features, plus albite, illite, mixed-layer illite/smectite, ferroan calcite, and dolomite formed in the meso-diagenetic realm (>70°C). Four of these features show spatial variability with respect to systems tracts. Infiltrated clays are concentrated in lowstand systems tracts (LST) and highstand systems tracts (HST) because accommodation space rose slow or fell during deposition of those sands, which led to prolonged sand body exposure on floodplain and ample opportunities for downward percolation of mud during flood events. Concentration of pseudomatrix (mud intraclasts) in HST and LST deposits resulted from floodplain erosion when base-level fell with decreasing accommodation space. Authigenic chlorite formed in the HST and transgressive systems tracts (TST) of the upper half of the Williams Fork Formation because volcanic clasts are abundant in that interval. Quartz overgrowths are more likely to exceed 7% in TST deposits for

  4. Dr. William Tumas - Associate Laboratory Director, Materials and Chemical

    Science.gov Websites

    Chemical Science and Technology Dr. William Tumas - Associate Laboratory Director, Materials and Chemical , technical direction, and workforce development of the materials and chemical science and technology , program management, and program execution. He joined NREL in December 2009 as Director of the Chemical and

  5. Williams working on the JAXA MS (Marangoni Surface) Experiment

    NASA Image and Video Library

    2009-11-05

    ISS021-E-020299 (5 Nov. 2009) --- NASA astronaut Jeffrey Williams, Expedition 21 flight engineer, works with Fluid Physics Experiment Facility/Marangoni Surface (FPEF MS) Core hardware in the Kibo laboratory of the International Space Station. The Marangoni convection experiment in the FPEF examines fluid tension flow in micro-G.

  6. Beyond Behaviour: Is Social Anxiety Low in Williams Syndrome?

    ERIC Educational Resources Information Center

    Dodd, Helen F.; Schniering, Carolyn A.; Porter, Melanie A.

    2009-01-01

    Individuals with Williams syndrome (WS) exhibit striking social behaviour that may be indicative of abnormally low social anxiety. The present research aimed to determine whether social anxiety is unusually low in WS and to replicate previous findings of increased generalised anxiety in WS using both parent and self report. Fifteen individuals…

  7. An Effective Leader in Higher Education: Charles William Eliot

    ERIC Educational Resources Information Center

    Elliott, Robert Wayne; Paton, Valerie Osland

    2014-01-01

    While responding to increasing calls for accountability, educational leaders are challenged continually by a myriad of complex tasks amidst the need to adapt their postsecondary institutions for responding to the changing needs of society. This review of literature examines comparisons between the leadership style of Charles William Eliot and the…

  8. Musicality Correlates with Sociability and Emotionality in Williams Syndrome

    ERIC Educational Resources Information Center

    Ng, Rowena; Lai, Philip; Levitin, Daniel J.; Bellugi, Ursula

    2013-01-01

    Williams syndrome (WS) is a neurogenetic developmental disorder characterized by peaks and valleys of cognitive abilities. One peak that has been understudied is the affinity that many individuals with WS have with music. It remains unknown whether their high levels of musical interest, skill, and expressivity are related to their sociable…

  9. 45. William E. Barrett, Photographer, August 1975. EARLY STEAM GENERATING ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    45. William E. Barrett, Photographer, August 1975. EARLY STEAM GENERATING UNIT USED TO PRODUCE ELECTRICITY FOR MANUFACTURING OPERATIONS AND FOR THE TOWN OF RAINELLE. STEAM ENGINE IS A HAMILTON CORLISS. - Meadow River Lumber Company, Highway 60, Rainelle, Greenbrier County, WV

  10. 40. William E. Barrett, Photographer, August 1975. ROOF OF POWERHOUSE ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    40. William E. Barrett, Photographer, August 1975. ROOF OF POWERHOUSE SHOWING HOPPERS FOR SAWDUST USED TO FIRE STEAM BOILERS. DUCTS AT LEFT LEAD FROM PLANNING MILL AND OTHER MANUFACTURING OPERATIONS. - Meadow River Lumber Company, Highway 60, Rainelle, Greenbrier County, WV

  11. Binding of Visual and Spatial Short-Term Memory in Williams Syndrome and Moderate Learning Disability

    ERIC Educational Resources Information Center

    Jarrold, Christopher; Phillips, Caroline; Baddeley, Alan D

    2007-01-01

    A main aim of this study was to test the claim that individuals with Williams syndrome have selectively impaired memory for spatial as opposed to visual information. The performance of 16 individuals with Williams syndrome (six males, 10 females; mean age 18y 7mo [SD 7y 6mo], range 9y 1mo-30y 7mo) on tests of short-term memory for item and…

  12. Williams conducts SWAB Sampling during Expedition 22

    NASA Image and Video Library

    2010-03-15

    ISS022-E-094369 (15 March 2010) --- NASA astronaut Jeffrey Williams, Expedition 22 commander, conducts a Surface, Water and Air Biocharacterization (SWAB) water sampling from the Potable Water Dispenser (PWD) in the Destiny laboratory of the International Space Station. SWAB uses advanced molecular techniques to comprehensively evaluate microbes onboard the space station, including pathogens (organisms that may cause disease). This study will allow an assessment of the risk of microbes to the crew and the spacecraft.

  13. Williams conducts SWAB Sampling during Expedition 22

    NASA Image and Video Library

    2010-03-15

    ISS022-E-094374 (15 March 2010) --- NASA astronaut Jeffrey Williams, Expedition 22 commander, conducts a Surface, Water and Air Biocharacterization (SWAB) water sampling from the Potable Water Dispenser (PWD) in the Destiny laboratory of the International Space Station. SWAB uses advanced molecular techniques to comprehensively evaluate microbes onboard the space station, including pathogens (organisms that may cause disease). This study will allow an assessment of the risk of microbes to the crew and the spacecraft.

  14. Early astronomy in America: the role of The College of William and Mary

    NASA Astrophysics Data System (ADS)

    Shy, Jeffery R.

    2002-06-01

    During the late eighteenth century, The College of William and Mary in Virginia, led by its president, Bishop James Madison, became a leading institution in the USA for the study of Natural Philosophy, and especially astronomy. In 1768, the College acquired scientific apparatus that had no equal in the colonies, and amont the items in this collection were astronomical instruments that were the finest in America. In 1778, Bishop Madison constructed what was certainly the first permanent observatory established anywhere in America. Madison's educational reforms and his personal involvement in the teaching of the natural sciences led to the first complete elective system of college courses in the USA. Unfortunately, the Revolutionary War devastated William and Mary and depleted its resources. Subsequently, the College was never able to achieve the great contributions to astronomy that may otherwise have been possible. Nevertheless, through its teaching programme, William and Mary contributed significantly to the education of many of the nation's early leaders, and it continues today as one of the foremost institutions of higher education in the USA.

  15. View of Williams during the PMDIS Experiment during STS-118/Expedition 15 Joint Operations

    NASA Image and Video Library

    2007-08-12

    S118-E-06912 (12 Aug. 2007) --- Astronaut Dave Williams, STS-118 mission specialist representing the Canadian Space Agency, works with the Perceptual Motor Deficits in Space (PMDIS) experiment in the Destiny laboratory of the International Space Station. The PMDIS experiment will measure the decline in hand-eye coordination of shuttle astronauts while on orbit. These measurements will be used to evaluate various mechanisms thought to be responsible for the decline. Astronaut Charlie Hobaugh, pilot, looks on as he floats above Williams.

  16. Autosomal dominant inheritance of Williams-Beuren syndrome in a father and son with haploinsufficiency for FKBP6.

    PubMed

    Metcalfe, Kay; Simeonov, Emil; Beckett, William; Donnai, Dian; Tassabehji, May

    2005-04-01

    Williams-Beuren syndrome (WBS) is a neurodevelopmental microdeletion disorder that usually occurs sporadically due to its location within a highly repetitive genomic region that is unstable and prone to unequal cross-over during meiosis. The consequential loss of chromosomal material includes approximately 1.5 Mb of DNA at 7q11.23. Whilst cases of dominant inheritance have been described in the literature, there have been few reports of molecular confirmation and none have carried out detailed genotyping. We describe a Bulgarian father and son with WBS detected by fluorescent in situ hybridisation (with an elastin gene probe) and loss of heterozygosity mapping using microsatellite markers located in the critical region. These individuals appear to have a common WBS heterozygous deletion, confirming the expected dominant transmission and adding to the few familial cases reported. The deletion includes the gene FKBP6 which has recently been shown to play a role in homologous chromosome pairing in meiosis and male fertility in mouse models. Homozygous Fkbp6 -/- male mice are infertile and our data suggests that haploinsufficiency for FKBP6 does not appear to preclude male fertility in WBS, although male infertility involving this gene has the potential to follow the mouse model as a human autosomal recessive condition.

  17. Brief Report: Repetitive Behaviour Profiles in Williams Syndrome: Cross Syndrome Comparisons with Prader-Willi and Down Syndromes

    ERIC Educational Resources Information Center

    Royston, R.; Oliver, C.; Moss, J.; Adams, D.; Berg, K.; Burbidge, C.; Howlin, P.; Nelson, L.; Stinton, C.; Waite, J.

    2018-01-01

    This study describes the profile of repetitive behaviour in individuals with Williams syndrome, utilising cross-syndrome comparisons with people with Prader-Willi and Down syndromes. The Repetitive Behaviour Questionnaire was administered to caregivers of adults with Williams (n = 96), Prader-Willi (n = 103) and Down (n = 78) syndromes. There were…

  18. 75 FR 30451 - Duke Energy Carolinas, LLC; Duke Energy Carolinas, LLC; William States Lee III Combined License...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-06-01

    ... Energy Carolinas, LLC; William States Lee III Combined License Application; Notice of Intent To Conduct a... environmental review of the William States Lee III Nuclear Station, Units 1 and 2 combined licenses application...-licensing/col/lee.html '' to `` http://www.nrc.gov/reactors/new-reactors/col/lee.html ''. Dated at Rockville...

  19. William Brennan and the Failed "Theory" of Actual Malice.

    ERIC Educational Resources Information Center

    Gillmor, Donald M.

    This paper contains an analysis of Justice William Brennan's Supreme Court opinions concerning cases on freedom of expression and his interpretations of Alexander Meiklejohn's theory of actual malice in cases of libel. Particular attention is paid to Brennan's landmark contribution to the law of libel, his opinion in "New York Times v.…

  20. The Darwinian Center to the Vision of William James.

    ERIC Educational Resources Information Center

    Bredo, Eric

    The essence of William James's vision can sometimes be hard to discover due to emotional volatility and exploratory impulsiveness. On the other hand, beneath James's apparent inconsistency was a constancy of purpose that can be easily underestimated. This paper argues that the center of James's vision lay in an interpretation of Darwinism. By…

  1. Climatic data for Williams Lake, Hubbard County, Minnesota, 1983

    USGS Publications Warehouse

    Sturrock, A.M.; Rosenberry, D.O.; Engelbrecht, L.G.; Gothard, W.A.; Winter, T.C.

    1984-01-01

    Research on the hydrology of Williams Lake, north-central Minnesota includes study of evaporation. Presented here are those climatic data needed for energy-budget and mass-transfer studies,including: water-surface temperature, dry-bulb and wet-bulb air temperatures, wind speed, precipitation, and solar radiation. Data are collected at raft and land stations.

  2. Astronaut William Fisher preparing to train in the WETF

    NASA Technical Reports Server (NTRS)

    1985-01-01

    Astronaut William Fisher is shown in his extravehicular mobility unit (EMU) preparing to train in the Weightless Environment Training Facility (WETF). He is wearing the communications carrier assembly but not the full helmet (32102); Reflections of the WETF can be seen on the closed visor of the EMU helmet Fiser is wearing (32103).

  3. Best Practices Case Study: Tommy Williams Homes -Gainesville, FL

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    none,

    2011-04-01

    Case study of Tommy Williams Homes who has continued to outsell the competition with sales increasing despite the recession thanks to a systems-engineering approach developed with DOE’s Building America that yields high energy efficiency, comfort, and indoor air quality. The company offers to pay buyers’ energy bills for the first year.

  4. Williams Syndrome and Memory: A Neuroanatomic and Cognitive Approach

    ERIC Educational Resources Information Center

    Sampaio, Adriana; Sousa, Nuno; Fernandez, Montse; Vasconcelos, Cristiana; Shenton, Martha E.; Goncalves, Oscar F.

    2010-01-01

    Williams Syndrome (WS) is described as displaying a dissociation within memory systems. As the integrity of hippocampal formation (HF) is determinant for memory performance, we examined HF volumes and its association with memory measures in a group of WS and in a typically development group. A significantly reduced intracranial content was found…

  5. Williams Syndrome: Daily Challenges and Positive Impact on the Family

    ERIC Educational Resources Information Center

    Scallan, Susan; Senior, Joyce; Reilly, Colin

    2011-01-01

    Background: Despite the distinctive physical, cognitive, personality and behavioural characteristics associated with Williams syndrome, few studies to date have examined parental experiences of raising a child with this genetic syndrome. Methods: This explorative pilot study employed predominantly qualitative methodologies via face-to-face…

  6. 2. Historic American Buildings Survey, William F. Winter, Jr., Photographer ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    2. Historic American Buildings Survey, William F. Winter, Jr., Photographer June 1931, SOUTH (LEFT) AND EAST SIDES, Gift of New York State Department of Education. - Shaker North Family Dwelling House (second), State Route 22 & U.S. Route 20, New Lebanon, Columbia County, NY

  7. 9. Historic American Buildings Survey, William F. Winter, Jr., Photographer ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    9. Historic American Buildings Survey, William F. Winter, Jr., Photographer Summer 1931, ATTIC WITH JOINING CHIMNEYS, Gift of New York State Department of Education. - Shaker North Family Dwelling House (second), State Route 22 & U.S. Route 20, New Lebanon, Columbia County, NY

  8. 3. Historic American Buildings Survey, William F. Winter, Jr., Photographer, ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    3. Historic American Buildings Survey, William F. Winter, Jr., Photographer, 1920's. GENERAL VIEW OF INNER 'YARD' LOOKING NORTH - CLOSE-UP, Gift of New York State Department of Education. - Shaker Church Family (General Views), Watervliet Shaker Road, Colonie Township, Watervliet, Albany County, NY

  9. 4. William Beardsley standing atop diversion dam. East cableway tower ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    4. William Beardsley standing atop diversion dam. East cableway tower and construction camp, Camp Dyer are visible in the foreground. Photographer James Dix Schuyler, 1903 Source: Schuyler report. - Waddell Dam, On Agua Fria River, 35 miles northwest of Phoenix, Phoenix, Maricopa County, AZ

  10. Williams uses computer in the U.S. Laboratory during Expedition 13

    NASA Image and Video Library

    2006-04-11

    ISS013-E-05853 (11 April 2006) --- Astronaut Jeffrey N. Williams, Expedition 13 NASA space station science officer and flight engineer, uses a computer in the Destiny laboratory of the International Space Station.

  11. Williams' paradox and the role of phenotypic plasticity in sexual systems.

    PubMed

    Leonard, Janet L

    2013-10-01

    As George Williams pointed out in 1975, although evolutionary explanations, based on selection acting on individuals, have been developed for the advantages of simultaneous hermaphroditism, sequential hermaphroditism and gonochorism, none of these evolutionary explanations adequately explains the current distribution of these sexual systems within the Metazoa (Williams' Paradox). As Williams further pointed out, the current distribution of sexual systems is explained largely by phylogeny. Since 1975, we have made a great deal of empirical and theoretical progress in understanding sexual systems. However, we still lack a theory that explains the current distribution of sexual systems in animals and we do not understand the evolutionary transitions between hermaphroditism and gonochorism. Empirical data, collected over the past 40 years, demonstrate that gender may have more phenotypic plasticity than was previously realized. We know that not only sequential hermaphrodites, but also simultaneous hermaphrodites have phenotypic plasticity that alters sex allocation in response to social and environmental conditions. A focus on phenotypic plasticity suggests that one sees a continuum in animals between genetically determined gonochorism on the one hand and simultaneous hermaphroditism on the other, with various types of sequential hermaphroditism and environmental sex determination as points along the spectrum. Here I suggest that perhaps the reason we have been unable to resolve Williams' Paradox is because the problem was not correctly framed. First, because, for example, simultaneous hermaphroditism provides reproductive assurance or dioecy ensures outcrossing does not mean that there are no other evolutionary paths that can provide adaptive responses to those selective pressures. Second, perhaps the question we need to ask is: What selective forces favor increased versus reduced phenotypic plasticity in gender expression? It is time to begin to look at the question

  12. Tectonic controls on large landslide complex: Williams Fork Mountains near Dillon, Colorado

    USGS Publications Warehouse

    Kellogg, K.S.

    2001-01-01

    An extensive (~ 25 km2) landslide complex covers a large area on the west side of the Williams Fork Mountains in central Colorado. The complex is deeply weathered and incised, and in most places geomorphic evidence of sliding (breakaways, hummocky topography, transverse ridges, and lobate distal zones) are no longer visible, indicating that the main mass of the slide has long been inactive. However, localized Holocene reactivation of the landslide deposits is common above the timberline (at about 3300 m) and locally at lower elevations. Clasts within the complex, as long as several tens of meters, are entirely of crystalline basement (Proterozoic gneiss and granitic rocks) from the hanging wall of the Laramide (Late Cretaceous to Early Tertiary), west-directed Williams Range thrust, which forms the western structural boundary of the Colorado Front Range. Late Cretaceous shale and sandstone compose most footwall rocks. The crystalline hanging-wall rocks are pervasively fractured or shattered, and alteration to clay minerals is locally well developed. Sackung structures (trenches or small-scale grabens and upslope-facing scarps) are common near the rounded crest of the range, suggesting gravitational spreading of the fractured rocks and oversteepening of the mountain flanks. Late Tertiary and Quaternary incision of the Blue River Valley, just west of the Williams Fork Mountains, contributed to the oversteepening. Major landslide movement is suspected during periods of deglaciation when abundant meltwater increased pore-water pressure in bedrock fractures. A fault-flexure model for the development of the widespread fracturing and weakening of the Proterozoic basement proposes that the surface of the Williams Range thrust contains a concave-downward flexure, the axis of which coincides approximately with the contact in the footwall between Proterozoic basement and mostly Cretaceous rocks. Movement of brittle, hanging-wall rocks through the flexure during Laramide

  13. William Herschel, the First Observational Cosmologist

    ScienceCinema

    Lemonick, Michael [Princeton University and Time Magazine, Princeton, New Jersey, United States

    2017-12-09

    In the late 1700s, a composer, orchestra director and soloist named William Herschel became fascinated with astronomy, and, having built his own reflecting telescope, went out in his garden in Bath, England, one night and discovered Uranus—the first planet in human history ever found by an individual. The feat earned him a lifetime pension from King George III. But Herschel considered the discovery to be relatively unimportant in comparison to his real work: understanding the composition, structure and evolution of the universe. In pursuing that work, he became the first observational cosmologist.

  14. Theory of mind in Williams syndrome assessed using a nonverbal task.

    PubMed

    Porter, Melanie A; Coltheart, Max; Langdon, Robyn

    2008-05-01

    This study examined Theory of Mind in Williams syndrome (WS) and in normal chronological age-matched and mental age-matched control groups, using a picture sequencing task. This task assesses understanding of pretence, intention and false belief, while controlling for social-script knowledge and physical cause-and-effect reasoning. The task was selected because it is entirely non-verbal, so that the WS individuals could not rely on their good verbal skills when performing the task. Results indicated a specific deficit in understanding of false belief within the WS group. There was also evidence of heterogeneity in the WS group, with the false belief impairment restricted to only a particular subgroup of WS individuals identified originally by Porter, M., & Coltheart, M. (2005). Cognitive heterogeneity in Williams syndrome. Developmental Neuropsychology, 27(2), 275-306.

  15. Williams syndrome as a model of genetically determined right-hemisphere dominance.

    PubMed

    Bogdanov, N N; Solonichenko, V G

    1997-01-01

    Studies were carried out on the dermatoglyphics (skin ridge marks) on the hands of children with Williams syndrome; this is an inherited disease with cardiovascular pathology and a characteristic facial phenotype ("elf" facies), along with specific mental and cognitive disturbances. The results suggest a characteristic dermatoglyphic type with the presence of complex whorls on the fingers and a clear predominance of marks of greater complexity on the left hand; this is a very rare trait in normal people and in those with other inherited nervous system disorders. The features of the dermatoglyphic pattern serve as a characteristic marker of a genetically determined state of the human central nervous system, and suggests directions for neurophysiological studies of children with Williams syndrome as a unique model for analysis of higher nervous function in humans.

  16. Climatic data for Williams Lake, Hubbard County, Minnesota, 1984

    USGS Publications Warehouse

    Sturrock, A.M.; Rosenberry, D.O.; Scarborough, J.L.; Winter, T.C.

    1986-01-01

    Research on the hydrology of Williams Lake, north-central Minnesota includes study of evaporation. Presented here are those climatic data needed for energy-budget and mass-transfer studies, including: water-surface temperature, dry-bulb and wet-bulb air temperatures, wind speed, precipitation, and solar and atmospheric radiation. Data are collected at raft and land stations.

  17. Climatic data for Williams Lake, Hubbard County, Minnesota, 1985

    USGS Publications Warehouse

    Sturrock, A.M.; Rosenberry, D.O.; Winter, T.C.

    1987-01-01

    Research on the hydrology of Williams Lake, north-central Minnesota includes study of evaporation. Presented here are those climatic data needed for energy-budget and mass-transfer studies, including: water-surface temperature, dry-bulb and wet-bulb air temperatures, wind speed, precipitation, and solar and atmospheric radiation. Data are collected at raft and land stations.

  18. 77 FR 32625 - William J. Stevenson, Estate of Lynn E. Stevenson, Black Canyon Bliss, LLC; Notice of Application...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-06-01

    ... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Project No. 8866-010] William J. Stevenson, Estate of Lynn E. Stevenson, Black Canyon Bliss, LLC; Notice of Application for Transfer of License, and Soliciting Comments and Motions To Intervene On April 23, 2012, William J. Stevenson, Estate...

  19. Williams uses communication equipment in the Airlock during Expedition 13

    NASA Image and Video Library

    2006-05-01

    ISS013-E-13327 (1 May 2006) --- Astronaut Jeffrey N. Williams, Expedition 13 NASA space station science officer and flight engineer, uses a communication system in the Quest Airlock of the International Space Station. Two Extravehicular Mobility Unit (EMU) spacesuits are visible in the background.

  20. Memory Abilities in Williams Syndrome: Dissociation or Developmental Delay Hypothesis?

    ERIC Educational Resources Information Center

    Sampaio, Adriana; Sousa, Nuno; Fernandez, Montse; Henriques, Margarida; Goncalves, Oscar F.

    2008-01-01

    Williams syndrome (WS) is a neurodevelopmental genetic disorder often described as being characterized by a dissociative cognitive architecture, in which profound impairments of visuo-spatial cognition contrast with relative preservation of linguistic, face recognition and auditory short-memory abilities. This asymmetric and dissociative cognition…

  1. 1. Historic American Buildings Survey, William F. Winter, Jr., Photographer, ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    1. Historic American Buildings Survey, William F. Winter, Jr., Photographer, 1920's or 1930's GENERAL VIEW OF WATERVLIET SHAKERS SOUTH FAMILY, Gift of New York State Department of Education. - Shaker South Family, General Views, Watervliet Shaker Road, Colonie Township, Watervliet, Albany County, NY

  2. 77 FR 29988 - Notice of Submission for OMB Review; Federal Student Aid; William D. Ford Federal Direct Loan...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-05-21

    ... benefits under the Department of Defense's Student Loan Repayment Program, or having a federal education... DEPARTMENT OF EDUCATION Notice of Submission for OMB Review; Federal Student Aid; William D. Ford... her William D. Ford Federal Direct Loan (Direct Loan) or Federal Family Education Loan (FFEL) Program...

  3. WE-G-213-00: History Symposium: Radiological Physics Pioneers: Roentgen and the AAPM Award Eponyms - William Coolidge, Edith Quimby, and Marvin Williams - Who Were They and What Did They Do?

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    NONE

    Roentgen and the Birth of Modern Medical Physics – Perry Sprawls Wilhelm Roentgen is well known for his discovery of x-radiation. What is less known and appreciated is his intensive research following the discovery to determine the characteristics of the “new kind of radiation” and demonstrate its great value for medical purposes. In this presentation we will imagine ourselves in Roentgen’s mind and follow his thinking, including questions and doubts, as he designs and conducts a series of innovative experiments that provided the foundation for the rapid growth of medical physics. Learning Objectives: Become familiar with the personal characteristics andmore » work of Prof. Roentgen that establishes him as an inspiring model for the medical physics profession. Observe the thought process and experiments that determined and demonstrated the comprehensive characteristics of x-radiation. The AAPM Award Eponyms: William D. Coolidge, Edith H. Quimby, and Marvin M.D. Williams - Who were they and what did they do? – Lawrence N. Rothenberg William David Coolidge (1873–1975) William Coolidge was born in Hudson, NY in 1873. He obtained his BS at the Massacusetts Institute of Technology in 1896. Coolidge then went to the University of Leipzig, Germany for graduate study with physicists Paul Drude and Gustave Wiedemann and received a Ph.D. in 1899. While in Germany he met Wilhelm Roentgen. Coolidge returned to the US to teach at MIT where he was associated with Arthur A. Noyes of the Chemistry Department, working on the electrical conductivity of aqueous solutions. Willis R. Whitney, under whom Coolidge had worked before going to Germany, became head of the newly formed General Electric Research Laboratory and he invited Coolidge to work with him. In 1905, Coolidge joined the staff of the GE laboratory and was associated with it for the remainder of his life. He developed ductile tungsten filaments to replace fragile carbon filaments as the material for electric

  4. Meet Marsha William: In Arms Reach Home Day Care

    ERIC Educational Resources Information Center

    Exchange: The Early Childhood Leaders' Magazine Since 1978, 2006

    2006-01-01

    Confident in what she knows, excited about what she's learning, eager to share her passion with others--this is Marsha Williams, director of In Arms Reach Home Day Care, Baltimore, Maryland. She enjoys being a family care provider, because with no more than eight children, she manages to give more individualized attention on a daily basis. She…

  5. Relationship of the Williams-Poulios and Manning-Rosen Potential Energy Models for Diatomic Molecules

    NASA Astrophysics Data System (ADS)

    Jia, Chun-Sheng; Liang, Guang-Chuan; Peng, Xiao-Long; Tang, Hong-Ming; Zhang, Lie-Hui

    2014-06-01

    By employing the dissociation energy and the equilibrium bond length for a diatomic molecule as explicit parameters, we generate an improved form of the Williams-Poulios potential energy model. It is found that the negative Williams-Poulios potential model is equivalent to the Manning-Rosen potential model for diatomic molecules. We observe that the Manning-Rosen potential is superior to the Morse potential in reproducing the interaction potential energy curves for the {{a}3 Σu+} state of the 6Li2 molecule and the {{X}1 sum+} state of the SiF+ molecule.

  6. Atypical Sleep Architecture and Altered EEG Spectra in Williams Syndrome

    ERIC Educational Resources Information Center

    Gombos, F.; Bodizs, R.; Kovacs, I.

    2011-01-01

    Background: Williams syndrome (WS) is a neurodevelopmental genetic disorder characterised by physical abnormalities and a distinctive cognitive profile with intellectual disabilities (IDs) and learning difficulties. Methods: In our study, nine adolescents and young adults with WS and 9 age- and sex-matched typically developing (TD) participants…

  7. 18. William E. Barrett, Photographer, August 1975. EXPOSED VIEW OF ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    18. William E. Barrett, Photographer, August 1975. EXPOSED VIEW OF LOWER PULLEYS OF LEFT-HAND MILL. LOWER LEFT IS BAND SAW PULLEY. UPPER LEFT IS TENSION WHEEL. LARGE PULLEY ON RIGHT IS DRIVE WHEEL FROM POWER SOURCE. - Meadow River Lumber Company, Highway 60, Rainelle, Greenbrier County, WV

  8. 33 CFR 167.1701 - In Prince William Sound: Precautionary areas.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ...: Precautionary areas. 167.1701 Section 167.1701 Navigation and Navigable Waters COAST GUARD, DEPARTMENT OF... Traffic Separation Schemes and Precautionary Areas Pacific West Coast § 167.1701 In Prince William Sound: Precautionary areas. (a) Cape Hinchinbrook. A precautionary area is established and is bounded by a line...

  9. 33 CFR 167.1701 - In Prince William Sound: Precautionary areas.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ...: Precautionary areas. 167.1701 Section 167.1701 Navigation and Navigable Waters COAST GUARD, DEPARTMENT OF... Traffic Separation Schemes and Precautionary Areas Pacific West Coast § 167.1701 In Prince William Sound: Precautionary areas. (a) Cape Hinchinbrook. A precautionary area is established and is bounded by a line...

  10. 33 CFR 167.1701 - In Prince William Sound: Precautionary areas.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ...: Precautionary areas. 167.1701 Section 167.1701 Navigation and Navigable Waters COAST GUARD, DEPARTMENT OF... Traffic Separation Schemes and Precautionary Areas Pacific West Coast § 167.1701 In Prince William Sound: Precautionary areas. (a) Cape Hinchinbrook. A precautionary area is established and is bounded by a line...

  11. 33 CFR 167.1701 - In Prince William Sound: Precautionary areas.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ...: Precautionary areas. 167.1701 Section 167.1701 Navigation and Navigable Waters COAST GUARD, DEPARTMENT OF... Traffic Separation Schemes and Precautionary Areas Pacific West Coast § 167.1701 In Prince William Sound: Precautionary areas. (a) Cape Hinchinbrook. A precautionary area is established and is bounded by a line...

  12. Dual-Task Processing as a Measure of Executive Function: A Comparison between Adults with Williams and Down Syndromes

    ERIC Educational Resources Information Center

    Kittler, Phyllis M.; Krinsky-McHale, Sharon J.; Devenny, Darlynne A.

    2008-01-01

    Behavioral phenotypes of individuals with Williams syndrome and individuals with Down syndrome have been contrasted in relation to short-term memory. People with Down syndrome are stronger visuospatially and those with Williams syndrome are stronger verbally. We examined short-term memory, then explored whether dual-task processing further…

  13. Identification of the TFII-I family target genes in the vertebrate genome.

    PubMed

    Chimge, Nyam-Osor; Makeyev, Aleksandr V; Ruddle, Frank H; Bayarsaihan, Dashzeveg

    2008-07-01

    GTF2I and GTF2IRD1 encode members of the TFII-I transcription factor family and are prime candidates in the Williams syndrome, a complex neurodevelopmental disorder. Our previous expression microarray studies implicated TFII-I proteins in the regulation of a number of genes critical in various aspects of cell physiology. Here, we combined bioinformatics and microarray results to identify TFII-I downstream targets in the vertebrate genome. These results were validated by chromatin immunoprecipitation and siRNA analysis. The collected evidence revealed the complexity of TFII-I-mediated processes that involve distinct regulatory networks. Altogether, these results lead to a better understanding of specific molecular events, some of which may be responsible for the Williams syndrome phenotype.

  14. Houston Intercontinental and William P. Hobby Air Traffic Control System Analysis

    DOT National Transportation Integrated Search

    1982-06-01

    This report provides a decription of the non-surveillance aspects of the FAA air traffic control facility operation at Houston Intercontinental and William P. Hobby Airports from teh air traffic controller's point of view. It includes photographs of ...

  15. BRIDGE BUILDER WILLIAM FLINN’S “CAMP & BRIDGE BUILDING OUTFIT”. INTERIOR ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    BRIDGE BUILDER WILLIAM FLINN’S “CAMP & BRIDGE BUILDING OUTFIT”. INTERIOR VIEW SHOWING LABORERS AT MEAL TIME. - Clear Fork of Brazos River Suspension Bridge, Spanning Clear Fork of Brazos River at County Route 179, Albany, Shackelford County, TX

  16. A Generalized Weizsacker-Williams Method Applied to Pion Production in Proton-Proton Collisions

    NASA Technical Reports Server (NTRS)

    Ahern, Sean C.; Poyser, William J.; Norbury, John W.; Tripathi, R. K.

    2002-01-01

    A new "Generalized" Weizsacker-Williams method (GWWM) is used to calculate approximate cross sections for relativistic peripheral proton-proton collisions. Instead of a mass less photon mediator, the method allows for the mediator to have mass for short range interactions. This method generalizes the Weizsacker-Williams method (WWM) from Coulomb interactions to GWWM for strong interactions. An elastic proton-proton cross section is calculated using GWWM with experimental data for the elastic p+p interaction, where the mass p+ is now the mediator. The resulting calculated cross sections is compared to existing data for the elastic proton-proton interaction. A good approximate fit is found between the data and the calculation.

  17. William Henry Bragg, man and scientist, Nobel Laureate and First Professor of Physics, University of Adelaide 1886-1909.

    PubMed

    Patterson, John; George, Robert

    2018-03-01

    In London, November 1915, a telegram was received at the home of William Henry Bragg from the secretary of the Academy of Science in Stockholm announcing the award of the Nobel Prize in Physics for "the analysis of crystal structures by means of X-rays". A second similar telegram was addressed to his 25 year old son William Lawrence Bragg (Jenkin, 2008). This article commemorates the centenary of that event and the unveiling of a bust of Sir William Bragg alongside that of his son, Sir Lawrence Bragg, on North Terrace in Adelaide where he spent 23 years of his early career. Copyright © 2018. Published by Elsevier Ltd.

  18. Ronald William Hodges, 1934-2017: Eminent Lepidopterist and Great Mentor

    USDA-ARS?s Scientific Manuscript database

    Dr. Ronald William Hodges (1934-2017) was an eminent lepidopterist and former Research Leader of the Systematic Entomology Laboratory from 1976 to 1979. He published on 117 genera (21 new), 806 species (351 new), 36 subfamilies (7 new), and 16 families (7 new) about gelechioid micromoths. He was awa...

  19. The Poetics of "Pattern Recognition": William Gibson's Shifting Technological Subject

    ERIC Educational Resources Information Center

    Wetmore, Alex

    2007-01-01

    William Gibson's 1984 cyberpunk novel "Neuromancer" continues to be a touchstone in cultural representations of the impact of new information and communication technologies on the self. As critics have noted, the posthumanist, capital-driven, urban landscape of "Neuromancer" resembles a Foucaultian vision of a panoptically engineered social space…

  20. The Interplay between Anxiety and Social Functioning in Williams Syndrome

    ERIC Educational Resources Information Center

    Riby, Deborah M.; Hanley, Mary; Kirk, Hannah; Clark, Fiona; Little, Katie; Fleck, Ruth; Janes, Emily; Kelso, Linzi; O'Kane, Fionnuala; Cole-Fletcher, Rachel; Allday, Marianne Hvistendahl; Hocking, Darren; Cornish, Kim; Rodgers, Jacqui

    2014-01-01

    The developmental disorder Williams syndrome (WS) has been associated with an atypical social profile of hyper-sociability and heightened social sensitivity across the developmental spectrum. In addition, previous research suggests that both children and adults with WS have a predisposition towards anxiety. The current research aimed to explore…

  1. 77 FR 29988 - Notice of Submission for OMB Review; Federal Student Aid; William D. Ford Federal Direct Loan...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-05-21

    ... borrowers in the William D. Ford Federal Direct Loan (Direct Loan) and Federal Family Education Loan (FFEL... DEPARTMENT OF EDUCATION Notice of Submission for OMB Review; Federal Student Aid; William D. Ford... mailed to [email protected] or mailed to U.S. Department of Education, 400 Maryland Avenue SW., LBJ...

  2. View of Mastracchio and Williams on EVA 1 during STS-118/Expedition 15 Joint Operations

    NASA Image and Video Library

    2007-08-11

    S118-E-06281 (11 Aug. 2007) --- Astronauts Rick Mastracchio (left) and Canadian Space Agency's Dave Williams, both STS-118 mission specialists, participate in the mission's first planned session of extravehicular activity (EVA), as construction continues on the International Space Station. During the 6-hour, 17-minute spacewalk Mastracchio and Williams attached the Starboard 5 (S5) segment of the station's truss, retracted the forward heat-rejecting radiator from the station's Port 6 (P6) truss, and performed several get-ahead tasks.

  3. Williams holds package of food in the SM during Expedition 13

    NASA Image and Video Library

    2006-06-01

    ISS013-E-29344 (1 June 2006) --- Astronaut Jeffrey N. Williams, Expedition 13 NASA space station science officer and flight engineer, holds a package of food near the galley in the Zvezda Service Module of the International Space Station.

  4. Expedition 21 FE Williams removes an EDV-U from the US WHC

    NASA Image and Video Library

    2009-10-30

    ISS021-E-016912 (30 Oct. 2009) --- NASA astronaut Jeffrey Williams, Expedition 21 flight engineer, works with the waste and hygiene compartment in the Destiny laboratory of the International Space Station.

  5. Love and Work: A Reading of John Williams' "Stoner"

    ERIC Educational Resources Information Center

    Frank, Jeff

    2017-01-01

    This article offers a close reading of the novel Stoner by John Williams. "Stoner," and not the countless reports and jeremiads on teaching, helps us find what we are searching for: a way to live--and talk about--teaching in a dignified and artful way. We need to seek out voices that remind, recall and reveal teaching for the beautifully…

  6. Astronaut William Readdy on flight deck wearing sun glasses

    NASA Image and Video Library

    1993-09-15

    STS051-16-012 (12-22 Sept 1993) --- On Discovery's forward flight deck, astronaut William F. Readdy, pilot, wears shades to block out bright sunshine. Much of the sunshine that normally would be coming through forward windows is blocked by an array of portable computers. Readdy was joined by four other NASA astronauts for almost ten full days in space.

  7. A Glimpse into the Life of William W. Brickman

    ERIC Educational Resources Information Center

    Brickman, Chaim Mann

    2010-01-01

    In this article, the author shares a little background on the life of William W. Brickman as his adoptive father. His father's parents immigrated to the United States from Jedwabne, northern Russian Poland around 1908. His father was born on June 30, 1913 in a tenement house located at 200 Eldridge Street on the Lower East Side of Manhattan. His…

  8. Pragmatic Language Impairment and Social Deficits in Williams Syndrome: A Comparison with Down's Syndrome and Specific Language Impairment

    ERIC Educational Resources Information Center

    Laws, Glynis; Bishop, Dorothy V. M.

    2003-01-01

    The social communication strength of individuals with Williams syndrome described by some researchers contrasts with the picture of social difficulties painted by others. To study the pragmatic aspects of language, social relationships and unusual interests in a group of children and adults with Williams syndrome, and to compare them with a group…

  9. Williams makes adjustment to strap in the SM during Expedition 13

    NASA Image and Video Library

    2006-04-18

    ISS013-E-08023 (18 April 2006) --- Astronaut Jeffrey N. Williams, Expedition 13 NASA space station science officer and flight engineer, prepares to exercise on the Treadmill Vibration Isolation System (TVIS) in the Zvezda Service Module of the International Space Station.

  10. Comprehension of Metaphor and Metonymy in Children with Williams Syndrome

    ERIC Educational Resources Information Center

    Annaz, Dagmara; Van Herwegen, Jo; Thomas, Michael; Fishman, Roza; Karmiloff-Smith, Annette; Rundblad, Gabriella

    2009-01-01

    Background: Figurative language, such as metaphor and metonymy, is very common in daily language use. Its underlying cognitive processes are sometimes viewed as lying at the interface of language and thought. Williams syndrome, which is a rare genetic developmental disorder, provides an opportunity to study this interface because individuals with…

  11. Perceptual Speech and Paralinguistic Skills of Adolescents with Williams Syndrome

    ERIC Educational Resources Information Center

    Hargrove, Patricia M.; Pittelko, Stephen; Fillingane, Evan; Rustman, Emily; Lund, Bonnie

    2013-01-01

    The purpose of this research was to compare selected speech and paralinguistic skills of speakers with Williams syndrome (WS) and typically developing peers and to demonstrate the feasibility of providing preexisting databases to students to facilitate graduate research. In a series of three studies, conversational samples of 12 adolescents with…

  12. How Flexible is the Use of Egocentric Versus Allocentric Frame of Reference in the Williams Syndrome Population?

    PubMed

    Heiz, J; Majerus, S; Barisnikov, K

    2017-09-28

    This study examined the spontaneous use of allocentric and egocentric frames of reference and their flexible use as a function of instructions. The computerized spatial reference task created by Heiz and Barisnikov (2015) was used. Participants had to choose a frame of reference according to three types of instructions: spontaneous, allocentric and egocentric. The performances of 16 Williams Syndrome participants between 10 and 41 years were compared to those of two control groups (chronological age and non-verbal intellectual ability). The majority of Williams Syndrome participants did not show a preference for a particular frame of reference. When explicitly inviting participants to use an allocentric frame of reference, all three groups showed an increased use of the allocentric frame of reference. At the same time, an important heterogeneity of type of frame of reference used by Williams Syndrome participants was observed. Results demonstrate that despite difficulties in the spontaneous use of allocentric and egocentric frames of reference, some Williams Syndrome participants show flexibility in the use of an allocentric frame of reference when an explicit instruction is provided. © The Author 2017. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  13. Williams works on computer in the U.S. Laboratory during Expedition 13

    NASA Image and Video Library

    2006-04-15

    ISS013-E-07975 (15 April 2006) --- Astronaut Jeffrey N. Williams, Expedition 13 NASA space station science officer and flight engineer, uses a computer in the Destiny laboratory of the International Space Station.

  14. Astronaut William Anders Official Portrait

    NASA Technical Reports Server (NTRS)

    1967-01-01

    This is the official NASA portrait of astronaut William Anders. Anders was commissioned in the air Force after graduation from the Naval Academy and served as a fighter pilot in all-weather interception squadrons of the Air Defense Command. Later he was responsible for technical management of nuclear power reactor shielding and radiation effects programs while at the Air Force Weapons Laboratory in New Mexico. In 1964, Anders was selected by the National Aeronautics and Space Administration (NASA) as an astronaut with responsibilities for dosimetry, radiation effects and environmental controls. He was backup pilot for the Gemini XI, Apollo 11 flights, and served as lunar module (LM) pilot for Apollo 8, the first lunar orbit mission in December 1968. He has logged more than 6,000 hours flying time.

  15. Examining Reports of Mental Health in Adults with Williams Syndrome

    ERIC Educational Resources Information Center

    Stinton, Chris; Tomlinson, Katie; Estes, Zachary

    2012-01-01

    Prior research suggests that individuals with Williams syndrome (WS) have a disposition towards anxiety. Information regarding this is typically derived from parents and carers. The perspectives of the individuals with WS are rarely included in research of this nature. We examined the mental health of 19 adults with WS using explicit (psychiatric…

  16. Personal Space Regulation in Williams Syndrome: The Effect of Familiarity

    ERIC Educational Resources Information Center

    Lough, Emma; Flynn, Emma; Riby, Deborah M.

    2016-01-01

    Personal space refers to a protective barrier that we strive to maintain around our body. We examined personal space regulation in young people with Williams syndrome (WS) and their typically developing, chronological age-matched peers using a parent report questionnaire and a stop-distance paradigm. Individuals with WS were reported by their…

  17. Human versus Non-Human Face Processing: Evidence from Williams Syndrome

    ERIC Educational Resources Information Center

    Santos, Andreia; Rosset, Delphine; Deruelle, Christine

    2009-01-01

    Increased motivation towards social stimuli in Williams syndrome (WS) led us to hypothesize that a face's human status would have greater impact than face's orientation on WS' face processing abilities. Twenty-nine individuals with WS were asked to categorize facial emotion expressions in real, human cartoon and non-human cartoon faces presented…

  18. Long-term memory for verbal and visual information in Down syndrome and Williams syndrome: performance on the Doors and People test.

    PubMed

    Jarrold, Christopher; Baddeley, Alan D; Phillips, Caroline

    2007-02-01

    Previous studies have suggested that Williams syndrome and Down syndrome may be associated with specific short-term memory deficits. Individuals with Williams syndrome perform relatively poorly on tests of visuo-spatial short-term memory and individuals with Down syndrome show a relative deficit on verbal short-term memory tasks. However, these patterns of impairments may reflect the impact of generally impaired visuo-spatial processing skills in Williams syndrome, and verbal abilities in Down syndrome. The current study explored this possibility by assessing long-term memory among 15 individuals with Williams syndrome and 20 individuals with Down syndrome using the Doors and People test, a battery which assesses recall and recognition of verbal and visual information. Individuals' performance was standardised for age and level of intellectual ability with reference to that shown by a sample of 110 typically developing children. The results showed that individuals with Down syndrome have no differential deficits in long-term memory for verbal information, implying that verbal short-term memory deficits in this population are relatively selective. Instead both individuals with Down syndrome and with Williams syndrome showed some evidence of relatively poor performance on tests of long-term memory for visual information. It is therefore possible that visuo-spatial short-term memory deficits that have previously been demonstrated in Williams syndrome may be secondary to more general problems in visuo-spatial processing in this population.

  19. Behavioral Profiles of Children with Williams Syndrome from Spain and the United States: Cross-Cultural Similarities and Differences

    ERIC Educational Resources Information Center

    Pérez-García, Débora; Brun-Gasca, Carme; Pérez-Jurado, Luis A.; Mervis, Carolyn B.

    2017-01-01

    To identify similarities and differences in the behavioral profile of children with Williams syndrome from Spain (n = 53) and the United States (n = 145), we asked parents of 6- to 14-year-olds with Williams syndrome to complete the Child Behavior Checklist 6-18. The distribution of raw scores was significantly higher for the Spanish sample than…

  20. Williams in the U.S. Laboratory during Expedition 13

    NASA Image and Video Library

    2006-08-17

    ISS013-E-67445 (17 Aug. 2006) --- Astronaut Jeffrey N. Williams, Expedition 13 NASA space station science officer and flight engineer, conducts an educational teleconference with the Boys and Girls Clubs of Middle Tennessee in Nashville, via Ku- and S-band in the Destiny laboratory of the International Space Station, with audio and video relayed to the Mission Control Center at Johnson Space Center.

  1. The Ffowcs Williams-Hawkings equation - Fifteen years of research

    NASA Technical Reports Server (NTRS)

    Farassat, F.

    1986-01-01

    The Ffowcs Williams-Hawkings equation governs the generation of sound in fluids in the presence of solid boundaries in motion. This equation is reviewed for situations where the linearization of the governing equations is allowed. In addition, research on the application of this equation to problems of aeroacoustic is briefly surveyed. Particular attention is given to the formulation of supersonic sources moving in uniform propeller-like motion.

  2. A Role for Transcription Factor GTF2IRD2 in Executive Function in Williams-Beuren Syndrome

    PubMed Central

    Porter, Melanie A.; Dobson-Stone, Carol; Kwok, John B. J.; Schofield, Peter R.; Beckett, William; Tassabehji, May

    2012-01-01

    Executive functions are amongst the most heritable cognitive traits with twin studies indicating a strong genetic origin. However genes associated with this domain are unknown. Our research into the neurodevelopmental disorder Williams-Beuren syndrome (WBS) has identified a gene within the causative recurrent 1.5/1.6 Mb heterozygous microdeletion on chromosome 7q11.23, which may be involved in executive functioning. Comparative genome array screening of 55 WBS patients revealed a larger ∼1.8 Mb microdeletion in 18% of cases, which results in the loss of an additional gene, the transcription factor GTF2IRD2. The GTF gene family of transcription factors (GTF2I, GTF2IRD1 and GTF2IRD2) are all highly expressed in the brain, and GTF2I and GTF2IRD1 are involved in the pathogenesis of the cognitive and behavioural phenotypes associated with WBS. A multi-level analysis of cognitive, behavioural and psychological functioning in WBS patients showed that those with slightly larger deletions encompassing GTF2IRD2 were significantly more cognitively impaired in the areas of spatial functioning, social reasoning, and cognitive flexibility (a form of executive functioning). They also displayed significantly more obsessions and externalizing behaviours, a likely manifestation of poor cognitive flexibility and executive dysfunction. We provide the first evidence for a role for GTF2IRD2 in higher-level (executive functioning) abilities and highlight the importance of integrating detailed molecular characterisation of patients with comprehensive neuropsychological profiling to uncover additional genotype-phenotype correlations. The identification of specific genes which contribute to executive function has important neuropsychological implications in the treatment of patients with conditions like WBS, and will allow further studies into their mechanism of action. PMID:23118870

  3. U.S. Rep. William Nelson drinking tea from shuttle beverage container

    NASA Technical Reports Server (NTRS)

    1985-01-01

    U.S. Rep. William Nelson of Florida tries drinking tea from a straw-equipped beverage dispenser in JSC's life sciences laboratory during a space food orientation session. The congressman is in early stages of training for a position on the STS 61-C mission.

  4. William Ellery Channing and Theodore Parker: A Study in Dialectical Enjoinment.

    ERIC Educational Resources Information Center

    Chandler, Daniel Ross

    This study discusses the preaching of William Ellery Channing and Theordore Parker as it contributed to the theological development of American intellectual life. Employing Cathcart's concept of "dialectical enjoinment," this study suggests that Channing and Parker exerted a restricted emphasis because communication with the larger…

  5. "Who Killed William Robinson?" Exploring a Nineteenth-Century Murder Online

    ERIC Educational Resources Information Center

    Sandwell, Ruth

    2004-01-01

    In 1996, the author and fellow-historian John Lutz set about creating a teaching tool for history that would acquaint students with primary documents and take full advantage of the brand-new technology of the World Wide Web. He launched the website, entitled "Who Killed William Robinson? Race, Justice and Settling the Land,"…

  6. How Executive Functions Are Related to Intelligence in Williams Syndrome

    ERIC Educational Resources Information Center

    Osorio, Ana; Cruz, Raquel; Sampaio, Adriana; Garayzabal, Elena; Martinez-Regueiro, Rocio; Goncalves, Oscar F.; Carracedo, Angel; Fernandez-Prieto, Montse

    2012-01-01

    Williams syndrome is characterized by impairments in executive functions (EFs). However, it remains unknown how distinct types of EFs relate to intelligence in this syndrome. The present study analyzed performance on working memory, inhibiting and shifting, and its links to IQ in a sample of 17 individuals with WS, and compared them with a group…

  7. Seasonal transport variations in the straits connecting Prince William Sound to the Gulf of Alaska

    NASA Astrophysics Data System (ADS)

    Halverson, Mark J.; Bélanger, Claude; Gay, Shelton M.

    2013-07-01

    Exchange of water between Prince William Sound and the Gulf of Alaska has a significant impact on its circulation and biological productivity. Current meter records from moored instruments in the two major straits connecting Prince William Sound to the Gulf of Alaska are analyzed to characterize the seasonal variations in water exchange. Eight individual deployments, each lasting for about 6 months, were made during the years 2005-2010. Two moorings were placed across each passage to account for horizontal flow variability. Monthly averaged, depth-integrated transport in winter is characterized by a strong barotropic inflow through Hinchinbrook Entrance and outflow through Montague Strait. The transport through each passage can reach 0.2Sv, which could replenish the volume of Prince William Sound in as little as 3 months. Depth-integrated transport is weaker and more variable in direction in summer than in winter, implying that Prince William sound is not always a simple flow-through system. Monthly transports range between -0.05 and 0.08Sv in each passage, and the corresponding flushing times exceed 1 year. The flow through both passages is highly baroclinic in the summer, so that the layer transport can be significant. For example, the deep inflow through Hinchinbrook Entrance can reach 0.05Sv, which would flush the deep regions of Prince William Sound (>400m) in only 23 days. The transport imbalance between Montague Strait and Hinchinbrook Entrance cannot be accounted for by considering other terms in a volume budget such as local freshwater input, meaning the imbalance is mostly a result of under-resolving the cross-strait flow variability. The magnitude of the monthly mean depth-integrated transport through Montague Strait and Hinchinbrook Entrance depends non-linearly on the shelf winds. Strong downwelling conditions, characteristic of the winter, drive inflow through Hinchinbrook Entrance, which is balanced by outflow through Montague Strait. Weak

  8. STS-79 Commander William Readdy arrives at SLF

    NASA Technical Reports Server (NTRS)

    1996-01-01

    STS-79 Commander William F. Readdy arrives at KSC's Shuttle Landing Facility with five fellow astronauts, ready to participate in the Terminal Countdown Demonstration Test (TCDT). The TCDT is a dress rehearsal for launch for the flight crew and launch team. Over the next several days, the astronauts will take part in training exercises at the launch pad that will culminate in a simulated launch countdown. The Space Shuttle Atlantis is being prepared for liftoff on STS-79 around September 12.

  9. The Rev. John Bracken v. the Visitors of William and Mary College: A Post-Revolutionary Problem in Visitatorial Jurisdiction.

    ERIC Educational Resources Information Center

    Bridge, J. W.

    1979-01-01

    Reforms in 1779 at the College of William and Mary caused a professor to be dismissed, after which he took legal action against the institution. It is concluded that English corporate law was abused in defending against the professor's action. (Journal availability: College of William and Mary, Williamsburg, VA 23185, $3.00.) (MSE)

  10. The Interplay between Executive Control and Motor Functioning in Williams Syndrome

    ERIC Educational Resources Information Center

    Hocking, Darren R.; Thomas, Daniel; Menant, Jasmine C.; Porter, Melanie A.; Smith, Stuart; Lord, Stephen R.; Cornish, Kim M.

    2013-01-01

    Previous studies suggest that individuals with Williams syndrome (WS), a rare genetically based neurodevelopmental disorder, show specific weaknesses in visual attention and response inhibition within the visuospatial domain. Here we examine the extent to which impairments in attentional control extend to the visuomotor domain using a…

  11. Adaptive Behavior and Problem Behavior in Young Children with Williams Syndrome

    ERIC Educational Resources Information Center

    Hahn, Laura J.; Fidler, Deborah J.; Hepburn, Susan L.

    2014-01-01

    The present study compares the adaptive behavior profile of 18 young children with Williams syndrome (WS) and a developmentally matched group of 19 children with developmental disabilities and examines the relationship between adaptive behavior and problem behaviors in WS. Parents completed the Vineland Adaptive Behavioral Scales--Interview…

  12. Limnological and geochemical survey of Williams Lake, Hubbard County, Minnesota

    USGS Publications Warehouse

    LaBaugh, J.W.; Groschen, G.E.; Winter, Thomas C.

    1981-01-01

    Calcium and bicarbonate represent more than 90 percent of the dissolved constituents in Williams Lake and the contiguous ground-water system. Major mineralogical constituents of the lake sediments are quartz, dolomite, and calcite. Marl is present only in the littoral zone of the lake. Organic sediments in the lake consist of loose organic floe and gyttja.

  13. Elicited Production of Relative Clauses in Children with Williams Syndrome

    ERIC Educational Resources Information Center

    Zukowski, Andrea

    2009-01-01

    Relative clauses have been implicated alternately as a strength and a weakness in the language of people with Williams Syndrome (WS). To clarify the facts, an elicited production test was administered to 10 people with WS (age 10-16 years), 10 typically developing children (age 4-7 years), and 12 typically developing adults. Nearly every WS…

  14. 21. Photographic copy of blueprint dated 1891; William R. Berger, ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    21. Photographic copy of blueprint dated 1891; William R. Berger, Chicago, architect; Original in collection of Rath drawings and blueprints owned by Waterloo Community Development Board, Waterloo, Iowa; DETAILS OF ICE CRIBS USED IN PACKINGHOUSE/CHILLING BUILDING IN THE ORIGINAL RATH PACKING PLANT - Rath Packing Company, Sycamore Street between Elm & Eighteenth Streets, Waterloo, Black Hawk County, IA

  15. The changing assessments of John Snow's and William Farr's cholera studies.

    PubMed

    Eyler, J M

    2001-01-01

    This article describes the epidemiological studies of cholera by two major British investigators of the mid-nineteenth century, John Snow and William Farr, and it asks why the assessments of their results by contemporaries was the reverse of our assessment today. In the 1840s and 1850s Farr's work was considered definitive, while Snow's was regarded as ingenious but flawed. Although Snow's conclusions ran contrary to the exceptations of his contemporaries, the major reservations about his cholera studies concerned his bold use of analogy, his thoroughgoing reductionism, and his willingness to ignore what seemed to be contrary evidence. Farr's electric use of current theories, his reliance multiple causation, and his discovery of a mathematical law to describe the outbreak in London in 1849 was much more convincing to his contemporaries. A major change in thinking about disease causation was needed before Snow's work could be widely accepted. William Farr's later studies contributed to that acceptance.

  16. William Herschel's 'Hole in the Sky' and the discovery of dark nebulae

    NASA Astrophysics Data System (ADS)

    Steinicke, Wolfgang

    2016-12-01

    In 1785 William Herschel published a paper in the Philosophical Transactions containing the remarkable section "An opening or hole". It describes an unusual vacant place in Scorpius. This matter falls into oblivion until Caroline Herschel initiated a correspondence with her nephew John in 1833. It contains Herschel's spectacular words "Hier ist wahrhaftig ein Loch im Himmel" ("Here truly is a hole in the sky"). About a hundred years later, Johann Georg Hagen, Director of the Vatican Observatory, presented a spectacular candidate for the 'hole', discovered in 1857 by Angelo Secchi in Sagittarius and later catalogued by Edward E. Barnard as the dark nebula B 86. Hagen's claim initiated a debate, mainly in the Journal of the British Astronomical Association, about the identity of Herschel's 'object'. Though things could be partly cleared up, unjustified claims still remain. This is mainly due to the fact that original sources were not consulted. A comprehensive study of the curious 'hole' is presented here. It covers major parts of the epochal astronomical work of William, Caroline and John Herschel. This includes a general study of 'vacant places', found by William Herschel and others, and the speculations about their nature, eventually leading to the finding that dark nebulae are due to absorbing interstellar matter. Some of the 'vacant places' could be identified in catalogues of dark nebulae and this leads to a 'Herschel Catalogue of Dark Nebulae' - the first historic catalogue of its kind.

  17. Comparison of CEAS and Williams-type models for spring wheat yields in North Dakota and Minnesota

    NASA Technical Reports Server (NTRS)

    Barnett, T. L. (Principal Investigator)

    1982-01-01

    The CEAS and Williams-type yield models are both based on multiple regression analysis of historical time series data at CRD level. The CEAS model develops a separate relation for each CRD; the Williams-type model pools CRD data to regional level (groups of similar CRDs). Basic variables considered in the analyses are USDA yield, monthly mean temperature, monthly precipitation, and variables derived from these. The Williams-type model also used soil texture and topographic information. Technological trend is represented in both by piecewise linear functions of year. Indicators of yield reliability obtained from a ten-year bootstrap test of each model (1970-1979) demonstrate that the models are very similar in performance in all respects. Both models are about equally objective, adequate, timely, simple, and inexpensive. Both consider scientific knowledge on a broad scale but not in detail. Neither provides a good current measure of modeled yield reliability. The CEAS model is considered very slightly preferable for AgRISTARS applications.

  18. Presence of autism, hyperserotonemia, and severe expressive language impairment in Williams-Beuren syndrome.

    PubMed

    Tordjman, Sylvie; Anderson, George M; Cohen, David; Kermarrec, Solenn; Carlier, Michèle; Touitou, Yvan; Saugier-Veber, Pascale; Lagneaux, Céline; Chevreuil, Claire; Verloes, Alain

    2013-08-23

    Deletion of the Williams-Beuren syndrome (WBS) critical region (WBSCR), at 7q11.23, causes a developmental disorder commonly characterized by hypersociability and excessive talkativeness and often considered the opposite behavioral phenotype to autism. Duplication of the WBSCR leads to severe delay in expressive language. Gene-dosage effects on language development at 7q11.23 have been hypothesized. Molecular characterization of the WBSCR was performed by fluorescence in situ hybridization and high-resolution single-nucleotide polymorphism array in two individuals with severe autism enrolled in a genetic study of autism who showed typical WBS facial dysmorphism on systematic clinical genetic examination. The serotonin transporter promoter polymorphism (5-HTTLPR, locus SLC6A4) was genotyped. Platelet serotonin levels and urinary 6-sulfatoxymelatonin excretion were measured. Behavioral and cognitive phenotypes were examined. The two patients had common WBSCR deletions between proximal and medial low copy repeat clusters, met diagnostic criteria for autism and displayed severe impairment in communication, including a total absence of expressive speech. Both patients carried the 5-HTTLPR ss genotype and exhibited platelet hyperserotonemia and low melatonin production. Our observations indicate that behaviors and neurochemical phenotypes typically associated with autism can occur in patients with common WBSCR deletions. The results raise intriguing questions about phenotypic heterogeneity in WBS and regarding genetic and/or environmental factors interacting with specific genes at 7q11.23 sensitive to dosage alterations that can influence the development of social communication skills. Thus, the influence of WBSCR genes on social communication expression might be dramatically modified by other genes, such as 5-HTTLPR, known to influence the severity of social communication impairments in autism, or by environmental factors, such as hyperserotonemia, given that

  19. Bringing inorganic chemistry to life with inspiration from R. J. P. Williams.

    PubMed

    Hill, H Allen O; Sadler, Peter J

    2016-03-01

    Our appreciation of the scholarly ideas and thinking of Bob Williams is illustrated here by a few of the areas in which he inspired us. His journey to bring inorganic chemistry to life began with an early interest in analytical chemistry, rationalising the relative stabilities of metal coordination complexes (The Irving-Williams Series), and elucidating the organometallic redox chemistry of vitamin B12. He (and Vallee) recognised that metal ions are in energised (entatic) states in proteins and enzymes, which themselves are dynamic structures of rods and springs. He played a key role in helping Rosenberg to pave the road toward the clinic for the anticancer drug cisplatin. He believed that evolution is not just dependent on DNA, but also on the metallome. Organisms and the environment are one system: does DNA code directly for all the essential elements of life?

  20. Viewing It Differently: Social Scene Perception in Williams Syndrome and Autism

    ERIC Educational Resources Information Center

    Riby, Deborah M.; Hancock, Peter J. B.

    2008-01-01

    The genetic disorder Williams syndrome (WS) is associated with a propulsion towards social stimuli and interactions with people. In contrast, the neuro-developmental disorder autism is characterised by social withdrawal and lack of interest in socially relevant information. Using eye-tracking techniques we investigate how individuals with these…

  1. Refining Behavioral Phenotypes: Personality-Motivation in Williams and Prader-Willi Syndromes.

    ERIC Educational Resources Information Center

    Dykens, Elisabeth M.; Rosner, Beth A.

    1999-01-01

    Personality-motivation was compared for age- and gender-matched individuals with Williams (n=35) or Prader-Willi (n=35) syndromes or mental retardation due to nonspecific causes (n=35). Each syndrome featured aberrant motivational profiles and similarities were found across groups in social interaction, anxiety, and orderliness. Recommendations…

  2. Sir William Osler's speech at Troy: a Trojan horse?

    PubMed

    Moran, Michael E

    2012-01-01

    Troy, New York, is a city of 55,000 people in upstate New York located along the Hudson River. A city of surprisingly rich cultural heritage, it was the home of New York state's first hospital outside New York City. The 50th anniversary celebration of Troy's hospital brought William Osler to the city as the keynote speaker. This speech, delivered on November 28, 1900, is one of Sir William's less well known addresses. Osler began his comments with Sir Thomas More's Utopia and talked at length about the hospital, its obligations, the influences it has upon the community, and the role of physicians and surgeons. He broached one of his old saws, the salary of attending physicians and their needed role in hospital management. His words were published in the diamond jubilee's records, but the hospital did not outlive its prominent guest professor, and it closed its doors in 1914. Just like the great historical city of Troy, New York's own Troy was on the brink of decline, and its hospital would be the first fatality. Therefore, it is almost prescient that the words of Osler, taken into historical context juxtaposed against the socioeconomic forces at work, are akin to the Greek's offering of a wooden edifice to end the Trojan War.

  3. Sir William Osler's speech at Troy: a Trojan horse?

    PubMed Central

    2012-01-01

    Troy, New York, is a city of 55,000 people in upstate New York located along the Hudson River. A city of surprisingly rich cultural heritage, it was the home of New York state's first hospital outside New York City. The 50th anniversary celebration of Troy's hospital brought William Osler to the city as the keynote speaker. This speech, delivered on November 28, 1900, is one of Sir William's less well known addresses. Osler began his comments with Sir Thomas More's Utopia and talked at length about the hospital, its obligations, the influences it has upon the community, and the role of physicians and surgeons. He broached one of his old saws, the salary of attending physicians and their needed role in hospital management. His words were published in the diamond jubilee's records, but the hospital did not outlive its prominent guest professor, and it closed its doors in 1914. Just like the great historical city of Troy, New York's own Troy was on the brink of decline, and its hospital would be the first fatality. Therefore, it is almost prescient that the words of Osler, taken into historical context juxtaposed against the socioeconomic forces at work, are akin to the Greek's offering of a wooden edifice to end the Trojan War. PMID:22275788

  4. Langley Deputy Chief Technologist Julie Williams-Byrd Speaks to Norfolk State University Students

    NASA Image and Video Library

    2018-02-06

    Deputy Chief Technologist Julie Williams-Byrd of NASA Langley Research Center speaks to Norfolk State University students following a “Hidden Figures to Modern Figures” event on February 6, 2018. (Credit: NASA)

  5. Meteor Beliefs Project: some meteoric imagery in the works of William Shakespeare

    NASA Astrophysics Data System (ADS)

    McBeath, A.; Gheorghe, A. D.

    2003-08-01

    Passages from three of William Shakespeare's plays are presented, illustrating some of the beliefs in meteors in 16th-17th century England. They also reflect earlier beliefs and information which it is known Shakespeare drew on in constructing his works.

  6. An Investigation into Semantic and Phonological Processing in Individuals with Williams Syndrome

    ERIC Educational Resources Information Center

    Lee, Cheryl S.; Binder, Katherine S.

    2014-01-01

    Purpose: The current study examined semantic and phonological processing in individuals with Williams syndrome (WS). Previous research in language processing in individuals with WS suggests a complex linguistic system characterized by "deviant" semantic organization and differential phonological processing. Method: Two experiments…

  7. Shoreline oiling conditions in Prince William Sound following the Exxon Valdez oil spill

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Neff, J.M.; Owens, E.H.; Stoker, S.W.

    1995-12-31

    Following the Exxon Valdez oil spill of March 24, 1989, in Prince William Sound, Alaska, Exxon conducted comprehensive, systematic shoreline surveys in cooperation with federal and state authorities to obtain information on the distribution and magnitude of shoreline oiling and to identify natural and cultural resources requiring special protection. Similar joint surveys were performed during the springs of 1990, 1991, and 1992 on all Prince william Sound and Gulf of Alaska shorelines that were suspected of having remnants of weathered oil and that would benefit from further cleanup. In the springs of 1990, 1991, and 1992, isolated pockets of subsurfacemore » oil were found, chiefly in small scattered zones in coarse cobble/boulder sediments in the upper intertidal or supratidal zones. In 1991, about one-third of the subdivisions in Prince William Sound with surface oil also contained some subsurface oil. The areal extent of this subsurface oil declined by nearly 70% between 1991 and 1992, from about 37,000 m{sup 2} to about 12,000 m{sup 2}. Moreover, where subsurface oil remained in 1992, it was present in lesser amounts. Rates of oil removal were greatest on coastal sections treated early in the spring and summer of 1989. Where shoreline treatment was delayed, the subsequent rate of removal of oil from the shore by natural processes was slower. 27 refs., 10 figs., 3 tabs.« less

  8. Astronaut William McArthur in medical experiment in SLS-2

    NASA Technical Reports Server (NTRS)

    1993-01-01

    Astronaut William S. McArthur, mission specialist, participates in an experiment using the rebreathing assembly and a gas analyzer mass spectrometer that investigates in-space distribution and movement of blood and gas in the pulmonary system. The data gathered during the two-week flight will be compared with results of tests performed on Earth to determine the changes that occur in pulmonary functions.

  9. Dr William Thornton (1759-1828) a savant of colonial America.

    PubMed

    Schneeberg, Norman G

    2006-08-01

    Dr William Thornton, an Edinburgh-trained physician, practised medicine sporadically in the British West Indies, the location of his birthplace, and in Philadelphia during post-revolutionary Colonial America. He is not well known to medical historians and 21st century physicians and is remembered principally as the amateur architect who designed the Capitol in Washington, DC and the Library Company of Philadelphia.

  10. Williams uses laptop computer in the U.S. Laboratory taken during Expedition 13

    NASA Image and Video Library

    2006-06-22

    ISS013-E-40000 (22 June 2006) --- Astronaut Jeffrey N. Williams, Expedition 13 NASA space station science officer and flight engineer, uses a computer in the Destiny laboratory of the International Space Station.

  11. Idiom Comprehension in French-Speaking Children and Adolescents with Williams' Syndrome

    ERIC Educational Resources Information Center

    Lacroix, Agnes; Aguert, Marc; Dardier, Virginie; Stojanovik, Vesna; Laval, Virginie

    2010-01-01

    This study looks at idiom comprehension by French-speaking people with Williams' syndrome (WS) and metapragmatic knowledge is examined. Idiomatic expressions are a nonliteral form of language where there is a considerable difference between what is said (literal interpretation) and what is meant (idiomatic interpretation). WS is characterized by a…

  12. William Golding's Iconoclastic Views about the Neanderthal Man in "The Inheritors"

    ERIC Educational Resources Information Center

    Youssef, Sayed Mohammed

    2016-01-01

    William Golding has been identified as a nonconformist whose opinions always go contrary to what is customarily accepted or established. This is shown in all his novels, more specifically "The Inheritors", in which he defies long established opinions held by anthropologists, historians, archaeologists as well as many others about the…

  13. Self Concept in People with Williams Syndrome and Prader-Willi Syndrome

    ERIC Educational Resources Information Center

    Plesa-Skwerer, Daniela; Sullivan, Kate; Joffre, Kristen; Tager-Flusberg, Helen

    2004-01-01

    This study explored self concepts in matched groups of adolescents and adults with Williams syndrome (WS) and Prader-Willi syndrome (PWS), using Damon and Hart's [Self-understanding in Childhood and Adolescence, Cambridge University Press, New York, 1988] semi-structured interview. The main findings were that the WS participants were more…

  14. Climatic data for Williams Lake, Hubbard County, Minnesota, 1982

    USGS Publications Warehouse

    Rosenberry, D.O.; Sturrock, A.M.; Scarborough, J.L.; Winter, T.C.

    1988-01-01

    Research on the hydrology of Williams Lake, north-central Minnesota includes study of evaporation. Those climatic data needed for energy budget and mass transfer studies are presented , including: water surface temperature, dry-bulb and wet-bulb air temperatures, wind speed, precipitation, and solar and atmospheric radiation. Some calculated values necessary for these studies are also presented, such as vapor pressure and Bowen-ratio values. Data are collected at raft and land stations.

  15. Climatic data for Williams Lake, Hubbard County, Minnesota, 1986

    USGS Publications Warehouse

    Rosenberry, D.O.; Sturrock, A.M.; Winter, T.C.

    1988-01-01

    Research on the hydrology of Williams Lake, north-central Minnesota includes study of evaporation. Presented here are those climatic data needed for energy-budget and mass-transfer studies, including: water-surface temperature, dry-bulb and wet-bulb air temperatures, wind speed, precipitation, and solar and atmospheric radiation. Some calculated values necessary for these studies, such as vapor pressure and Bowen ratio numbers, also are presented. Data are collected at raft and land stations.

  16. Sir William Johnson: Lessons-Learned from an Irregular Warfighter

    DTIC Science & Technology

    2011-06-01

    Meath, Ireland. 1 His Catholic father and Anglican mother made William an outsider to both religious groups, at a time when spiritual affiliation in...this sometimes deadly vaccination attests to the faith and confidence they had in him.34 Along with his prominent status in cofonial· society, Johnson...especially when it came to the upbringing of their children . Johnson’s humility, and willingness to acknowledge the many positive aspects of the

  17. Astronaut William Pogue using Skylab Viewfinder Tracking System experiment

    NASA Image and Video Library

    1973-09-10

    S73-32854 (10 Sept. 1973) --- Astronaut William R. Pogue, Skylab 4 pilot, uses the Skylab Viewfinder Tracking System (S191 experiment) during a training exercise in the Multiple Docking Adapter (MDA) one-G trainer at Johnson Space Center. In the background is astronaut Gerald P. Carr, seated at the control panel for the Earth Resources Experiments Package (EREP). Carr is Skylab 4 crew commander, and Gibson is science pilot. Photo credit: NASA

  18. Centennial ties: Harvey Cushing (1869-1939) and William Osler (1849-1919) on Andreas Vesalius (1514-1564).

    PubMed

    Toodayan, Nadeem

    2017-08-01

    Andreas Vesalius is often regarded as the founding father of modern anatomical study. The quincentennial anniversary of his birth - 31 December 2014 - has been very widely commemorated, and it is the purpose of this article to contrast these celebrations with what happened during the Vesalius quatercentenary year of 1914. More specifically, we look at how Vesalius was perceived a century ago by examining his influence on two of western medicine's most iconic gentlemen - Harvey Williams Cushing (1869-1939) and his larger than life mentor, Sir William Osler (1849-1919).

  19. STS-116 crewmembers Williams and Fuglesang prepare to open food packages on the MDDK on Space Shuttle Discovery

    NASA Image and Video Library

    2006-12-10

    S116-E-05289 (10 Dec. 2006) --- Astronaut Sunita L. Williams (right) and European Space Agency (ESA) astronaut Christer Fuglesang, both STS-116 mission specialists, enjoy a light moment as they prepare to open food packages on the middeck of Space Shuttle Discovery. Williams will join the Expedition 14 crew as flight engineer after she enters the International Space Station. Docking of the two spacecraft will occur on Dec. 11.

  20. Williams syndrome starts making sense

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Ashkenas, J.

    1996-10-01

    1996 may be marked as a transitional year in the study of Williams syndrome (WS), when the causes of this complex condition and a practical way to investigate began to come into focus. WS presents a remarkable collection of symptoms that affect blood vessels, growth, intelligence, and behavior. WS commonly leads to infantile hypercalcemia, retardation of growth, prematurely wrinkled skin, supraventricular aortic stenosis (SVAS), and sensitivity to loud noise. Children with this condition are often mentally retarded, with distinctive {open_quotes}elfin{close_quotes} facial features, a hoarse voice, and an {open_quotes}engaging{close_quotes} personality. Their cognitive deficits may be minimal or profound but typically involvemore » a specific pattern of strengths and weaknesses, with better-than-average face recognition but little ability to recognize how parts of patterns that they see fit into a whole. 36 refs.« less