Inversion (X)(p11.4q22) associated with Norrie disease in a four generation family.

PubMed

Pettenati, M J; Rao, P N; Weaver, R G; Thomas, I T; McMahan, M R

1993-03-01

We report on a 4-generation family in which Norrie disease occurs together with a pericentric inversion of the X chromosome in all affected males and carrier females. The breakpoint in the short arm of the X chromosome appears to be at the purported location of the Norrie disease gene. This is the second report of an association between Norrie disease and a chromosome aberration involving Xp11, and the first report of a specific gene disruption, thus physical gene location, due to a pericentric chromosome inversion.

The ocular pathology of Norrie disease in a fetus of 11 weeks' gestational age.

PubMed

Parsons, M A; Curtis, D; Blank, C E; Hughes, H N; McCartney, A C

1992-01-01

The ocular pathology of Norrie disease was studied for the first time in a fetus of 11 weeks' gestation, following prenatal diagnosis using genetic markers for Norrie disease and elective abortion. The eyes were histologically normal, with no evidence of primary neuroectodermal maldevelopment of the retina, previously postulated to be the cause of the ocular changes. We believe that the retinal and other manifestations of Norrie disease are the result of a primary abnormality of vascular proliferation, probably in relation to persistent hyperplastic primary vitreous after approximately 14 weeks' gestation. We postulate that the ocular and otological effects of Norrie disease may be due to a genetically mediated abnormality of secretion of, or sensitivity to, angiogenic growth factors at endodermal-neuroectodermal interfaces during fetal and postnatal development.

A novel missense Norrie disease mutation associated with a severe ocular phenotype.

PubMed

Khan, Arif O; Shamsi, Farrukh A; Al-Saif, Amr; Kambouris, Marios

2004-01-01

Clinical findings and pedigree analysis led to the diagnosis of severe Norrie disease in two brothers. DNA sequencing demonstrated a novel missense mutation (703G>T) that significantly alters predicted protein structure. Less severe retinal developmental disease may be associated with milder mutations in the Norrie disease gene.

In-utero diagnosis of Norrie disease by ultrasonography.

PubMed

Redmond, R M; Vaughan, J I; Jay, M; Jay, B

1993-03-01

Obstetric ultrasonography of an obligate Norrie disease carrier revealed bilateral retinal detachments in a third trimester male fetus. Postnatal examination confirmed the diagnosis of Norrie disease. DNA linkage analysis with the markers L1.28 and MAO had been uninformative for this family. This report suggests that retinal detachment occurs late in the gestation of the affected fetus.

Audiologic features of Norrie disease.

PubMed

Halpin, Chris; Owen, Grace; Gutiérrez-Espeleta, Gustavo A; Sims, Katherine; Rehm, Heidi L

2005-07-01

Norrie disease is an X-linked recessive disorder in which patients are born blind and develop sensory hearing loss in adolescence. The hearing loss associated with Norrie disease has been shown in a genetically altered knockout mouse to involve dysfunction of the stria vascularis; most other structures are preserved until the later stages of the disease. The objective of this study was to characterize the audiologic phenotype of Norrie disease for comparison with the pathophysiologic mechanism. The design combined two series of clinical audiologic evaluations, with special attention to speech intelligibility. The audiologic results for 12 affected individuals and 10 carriers show that patients with Norrie disease retain high speech intelligibility scores even when the threshold loss is severe. The cochlear mechanism-- failure of the stria vascularis-- accounts for some of the higher values in the wide distribution of speech scores in cases with similar pure tone audiograms.

[Norrie syndrome: identification of carriers by segregation analysis with flanking DNA markers].

PubMed

Körner, J; Uhlhaas, S; Neugebauer, M; Gal, A

1989-01-01

Norrie disease is an X-linked recessive disorder. Affected males present with congenital blindness. Additionally, hearing loss and psychotic behavior may occur at any time. Since carriers are clinically healthy, they can only be identified by genetic means. Daughters of carriers or sisters of affected males have an à priori 50% risk of being carriers themselves. Close linkage has been found between the Norrie disease locus (NDP) and the DNA locus DXS7 mapped to Xp11.3. For genetic counselling, this linkage relationship allows carriers of the disease to be identified in informative families. We describe a large pedigree with Norrie disease. Segregation analysis was carried out with DXS7 and a second flanking marker, DXS255, both linked to NDP. In this way, three females at risk were identified who had a high probability of being carriers for Norrie disease.

Origins of water and solutes in and north of the Norris-Mammoth Corridor, Yellowstone National Park

USGS Publications Warehouse

Kharaka, Yousif; Mariner, Robert; Ambats, Gil; Evans, William; White, Lloyd; Bullen, Thomas; Kennedy, B. Mack

1990-01-01

This study was initiated to investigate the impacts of geothermal development in the Corwin Springs Known Geothermal Resources Area (KGRA) on the hydrothermal features of Yellowstone National Park. To determine possible hydrogeochemical connections, we used the diagnostic stable and radioactive isotopes of several elements, and the chemical and gas compositions of thermal and cold waters from the Norris-Mammoth Corridor and areas north of the Park. The investigations were particularly comprehensive in the Mammoth Hot Springs area, Corwin Springs KGRA, and Chico Hot Springs. The geochemical tools used are still subject to uncertainties of 1 - 5%. Preliminary interpretation of the data, especially the ??D and ??18O values of water, 87Sr/86Sr ratios, ??11B values, composition and isotopes of noble gases and several conservative chemical species would indicate that the waters from Mammoth Hot Springs and La Duke Spring area have evolved chemically and isotopically by reactions with different rock types, and are probably not directly connected. These data indicate that a component (<20%) of water in Bear Creek Springs may be derived from the Mammoth system.

The Geology and Remarkable Thermal Activity of Norris Geyser Basin, Yellowstone National Park, Wyoming

USGS Publications Warehouse

White, Donald Edward; Hutchinson, Roderick A.; Keith, Terry E.C.

1988-01-01

Norris Geyser Basin, normally shortened to Norris Basin, is adjacent to the north rim of the Yellowstone caldera at the common intersection of the caldera rim and the Norris-Mammoth Corridor, a zone of faults, volcanic vents, and thermal activity that strikes north from the caldera rim to Mammoth Hot Springs. An east-west fault zone terminates the Gallatin Range at its southern end and extends from Hebgen Lake, west of the park, to Norris Basin. No local evidence exists at the surface in Norris Basin for the two oldest Yellowstone volcanic caldera cycles (~2.0 and 1.3 m.y.B.P.). The third and youngest cycle formed the Yellowstone caldera, which erupted the 600,000-year-old Lava Creek Tuff. No evidence is preserved of hydrothermal activity near Norris Basin during the first 300,000.years after the caldera collapse. Glaciation probably removed most of the early evidence, but erratics of hot-spring sinter that had been converted diagenetically to extremely hard, resistant chalcedonic sinter are present as cobbles in and on some moraines and till from the last two glacial stages, here correlated with the early and late stages of the Pinedale glaciation <150,000 years B.P.). Indirect evidence for the oldest hydrothermal system at Norris Basin indicates an age probably older than both stages of Pinedale glaciation. Stream deposits consisting mainly of rounded quartz phenocrysts of the Lava Creek Tuff were subaerial, perhaps in part windblown and redeposited by streams. A few small rounded pebbles are interpreted as chalcedonic sinter of a still older cycle. None of these are precisely dated but are unlikely to be more than 150,000 to 200,000 years old. ...Most studies of active hydrothermal areas have noted chemical differences in fluids and alteration products but have given little attention to differences and models to explain evolution in types. This report, in contrast, emphasizes the kinds of changes in vents and their changing chemical types of waters and then provides models for explaining these differences. Norris Basin is probably not an independent volcanic-hydrothermal system. The basin and nearby acid-leached areas (from oxidation of H2S-enriched vapor) are best considered as parts of the same system, extending from Norris Basin to Roaring Mountain and possibly to Mammoth. If so, are they parts of a single large system centered within the Yellowstone caldera, or are Norris Basin and the nearby altered areas both parts of one or more young independent corridor systems confined, at least in the shallow crust, to the Norris-Mammoth Corridor? Tentatively, we favor the latter relation, probably having evolved in the past ~300,000 years. A model for large, long-lived, volcanic-hydrothermal activity is also suggested, involving all of the crust and upper mantle and using much recent geophysical data bearing on crust-mantle interrelations. Our model for large systems is much superior to previous suggestions for explaining continuing hydrothermal activity over hundreds of thousands of years, but is less attractive for the smaller nonhomogenized volcanic system actually favored here for the Norris-Mammoth Corridor.

Isolated Norrie disease in a female caused by a balanced translocation t(X,6).

PubMed

Meire, F M; Lafaut, B A; Speleman, F; Hanssens, M

1998-12-01

This is the second report of Norrie disease in a female patient with a de-novo balanced translocation t(X,6) with breakpoint at the location of the Norrie gene. At the age of 3 months, a girl was referred for suspected congenital glaucoma. The right eye was microphthalmic and ultrasonography was compatible with persistent hyperplasia of the primary vitreous. The left eye was also microphthalmic. The left cornea was larger than the right. The anterior chamber was virtual and leukocoria was evident. The eye felt hard digitally. Ultrasonography indicated an organized retinal detachment. The pathologic findings are reported and are compatible with Norrie disease.

Identification of novel missense mutations in the Norrie disease gene associated with one X-linked and four sporadic cases of familial exudative vitreoretinopathy.

PubMed

Shastry, B S; Hejtmancik, J F; Trese, M T

1997-01-01

X-linked Familial Exudative Vitreoretinopathy (XLFEVR) is a hereditary eye disorder that affects both the retina and the vitreous body. It is characterized by an abnormal vascularization of the peripheral retina. It has been previously shown by linkage and candidate gene analysis that XLFEVR and Norrie disease are allelic. In this report we describe four novel mutations (R41K, H42R, K58N, and Y120C) in the Norrie disease gene associated with one X-linked and four sporadic cases of FEVR. One mutation (H42R) was found to be segregating with the disease in three generations (X-linked family), and the others are sporadic. These sequence alterations changed the encoded amino acids in the Norrie disease protein and were not found in 17 unaffected family members or in 36 randomly selected normal individuals. This study provides additional evidence that mutations in the same gene can result in FEVR and Norrie disease. It also demonstrates that it may be beneficial for clinical diagnosis to screen for mutations in the Norrie disease gene in sporadic FEVR cases.

Ocular findings associated with a Cys39Arg mutation in the Norrie disease gene.

PubMed

Joos, K M; Kimura, A E; Vandenburgh, K; Bartley, J A; Stone, E M

1994-12-01

To diagnose the carriers and noncarriers in a family affected with Norrie disease based on molecular analysis. Family members from three generations, including one affected patient, two obligate carriers, one carrier identified with linkage analysis, one noncarrier identified with linkage analysis, and one female family member with indeterminate carrier status, were examined clinically and electrophysiologically. Linkage analysis had previously failed to determine the carrier status of one female family member in the third generation. Blood samples were screened for mutations in the Norrie disease gene with single-strand conformation polymorphism analysis. The mutation was characterized by dideoxy-termination sequencing. Ophthalmoscopy and electroretinographic examination failed to detect the carrier state. The affected individuals and carriers in this family were found to have a transition from thymidine to cytosine in the first nucleotide of codon 39 of the Norrie disease gene, causing a cysteine-to-arginine mutation. Single-strand conformation polymorphism analysis identified a patient of indeterminate status (by linkage) to be a noncarrier of Norrie disease. Ophthalmoscopy and electroretinography could not identify carriers of this Norrie disease mutation. Single-strand conformation polymorphism analysis was more sensitive and specific than linkage analysis in identifying carriers in this family.

A novel missense NDP mutation [p.(Cys93Arg)] with a manifesting carrier in an austrian family with Norrie disease.

PubMed

Parzefall, Thomas; Lucas, Trevor; Ritter, Markus; Ludwig, Martin; Ramsebner, Reinhard; Frohne, Alexandra; Schöfer, Christian; Hengstschläger, Markus; Frei, Klemens

2014-01-01

Norrie disease is a rare, X-linked genetic syndrome characterized by combined congenital blindness and progressive hearing impairment. Norrie disease is caused by alterations in the NDP gene encoding the growth factor norrin that plays a key role in vascular development and stabilization of the eye, inner ear and brain. We identified a family with 3 affected deafblind males and a single female carrier presenting with a serous retinal detachment but normal hearing. Genetic analysis revealed a novel c.277T>C missense mutation causing the substitution of a hydrophobic cysteine to a hydrophilic arginine [p.(Cys93Arg)] within the highly conserved cysteine knot domain of the norrin protein. These results should expand the scope for amniocentesis and genetic testing for Norrie disease which is gaining in importance due to novel postnatal therapeutic concepts to alleviate the devastating retinal symptoms of Norrie disease. © 2014 S. Karger AG, Basel.

Norrie disease resulting from a gene deletion: clinical features and DNA studies.

PubMed

Donnai, D; Mountford, R C; Read, A P

1988-02-01

We describe a family in which two boys with Norrie disease have a deletion of the DXS7 locus. The deletion does not extend as far distally as the OTC or DXS84 loci. A full clinical description of the patients is given to help establish the range of manifestations of Norrie disease. There is no evidence of any other X linked disease in our patients.

Usual source of care and the quality of medical care experiences: a cross-sectional survey of patients from a Taiwanese community.

PubMed

Tsai, Jenna; Shi, Leiyu; Yu, Wei-Lung; Lebrun, Lydie A

2010-07-01

This study used a recent patient survey to examine the relationship between having a usual source of care (USC) and the quality of ambulatory medical care experiences in Taiwan, where there is universal health insurance coverage. The study design was a cross-sectional survey of 879 patients in Taichung County, Taiwan. Children and adults visiting hospital-based physicians were included. Quality of care was measured using items from the Primary Care Assessment Tool (PCAT), representing 7 ambulatory medical care domains: first contact (ie, access and utilization), longitudinality (ie, ongoing care), coordination (ie, referrals and information systems), comprehensiveness (ie, services available and provided), family centeredness, community orientation, and cultural competence. USC was defined based on responses to 3 survey items from the PCAT. Having a USC was significantly associated with higher quality of medical care experiences. Specifically, having a USC was associated with improved accessibility and utilization, ongoing care, coordination of referrals, and healthcare providers' family centeredness and cultural competence. However, having a USC was not strongly related with comprehensiveness of services, coordination of information systems, or healthcare providers' community orientation. In a region with universal health insurance, patients with a USC reported higher quality of medical care experiences compared with those without a USC. Beyond the provision of health insurance coverage, efforts to improve quality of care should include policies promoting USC.

Norrie disease resulting from a gene deletion: clinical features and DNA studies.

PubMed Central

Donnai, D; Mountford, R C; Read, A P

1988-01-01

We describe a family in which two boys with Norrie disease have a deletion of the DXS7 locus. The deletion does not extend as far distally as the OTC or DXS84 loci. A full clinical description of the patients is given to help establish the range of manifestations of Norrie disease. There is no evidence of any other X linked disease in our patients. Images PMID:3162283

Prenatal exclusion of Norrie disease with flanking DNA markers.

PubMed

Gal, A; Uhlhaas, S; Glaser, D; Grimm, T

1988-10-01

Three polymorphic DNA markers linked to the locus of Norrie disease were used for indirect genotype analysis in a ten-wk-old fetus at risk for the disease. When haplotypes of the family members and the estimated recombination frequency between Norrie gene and each of the DNA marker loci DXS7, DXS84, and DXS146 were taken into account, the risk that the fetus had inherited the mutation was about 1%.

30 CFR 875.16 - Exclusion of certain noncoal reclamation sites.

Code of Federal Regulations, 2010 CFR

2010-07-01

... Radiation Control Act of 1978 (42 U.S.C. 7901 et seq.) or that have been listed for remedial action under the Comprehensive Environmental Response Compensation and Liability Act of 1980 (42 U.S.C. 9601 et seq... Uranium Mill Tailings Radiation Control Act of 1978 (42 U.S.C. 7901 et seq.) or that have been listed for...

Retinoschisislike alterations in the mouse eye caused by gene targeting of the Norrie disease gene.

PubMed

Ruether, K; van de Pol, D; Jaissle, G; Berger, W; Tornow, R P; Zrenner, E

1997-03-01

To investigate the retinal function and morphology of mice carrying a replacement mutation in exon 2 of the Norrie disease gene. Recently, Norrie disease mutant mice have been generated using gene targeting technology. The mutation removes the 56 N-terminal amino acids of the Norrie gene product. Ganzfeld electroretinograms (ERGs) were obtained in five animals hemizygous or homozygous for the mutant gene and in three female animals heterozygous for the mutant gene. As controls, three males carrying the wild-type gene were examined. Electroretinogram testing included rod a- and b-wave V-log I functions, oscillatory potentials, and cone responses. The fundus morphology has been visualized by scanning laser ophthalmoscopy. Rod and cone ERG responses and fundus morphology were not significantly different among female heterozygotes and wild-type mice. In contrast, the hemizygous mice displayed a severe loss of ERG b-wave, leading to a negatively shaped scotopic ERG and a marked reduction of oscillatory potentials. The a-wave was normal at low intensities, and only with brighter flashes was there a moderate amplitude loss. Cone amplitudes were barely recordable in the gene-targeted males. Ophthalmoscopy revealed snowflakelike vitreal changes, retinoschisis, and pigment epithelium irregularities in hemizygotes and homozygotes, but no changes in female heterozygotes. The negatively shaped scotopic ERG in male mice with a Norrie disease gene mutation probably was caused by retinoschisis. Pigment epithelial changes and degenerations of the outer retina are relatively mild. These findings may be a clue to the embryonal retinoschisislike pathogenesis of Norrie disease in humans or it may indicate a different expression of the Norrie disease gene defect in mice compared to that in humans.

Laser photocoagulation at birth prevents blindness in Norrie's disease diagnosed using amniocentesis.

PubMed

Chow, Clement C; Kiernan, Daniel F; Chau, Felix Y; Blair, Michael P; Ticho, Benjamin H; Galasso, John M; Shapiro, Michael J

2010-12-01

To report the first case of prophylactic laser treatment to prevent blindness in a patient who was diagnosed with Norrie's disease by genetic testing with amniocentesis. Case report. A 2-year-old white boy with Norrie's disease. A 37-week gestational age male with a family history of Norrie's disease was born via Cesarean section after the mother had undergone prenatal amniocentesis fetal-genetic testing at 23 weeks of gestation. A C520T (nonsense) mutation was found in the Norrie's disease gene. After examination under anesthesia confirmed the diagnosis on the first day of life, laser photocoagulation was applied to the avascular retina bilaterally. The patient was followed closely by ophthalmology, pediatrics, and occupational therapy departments. Functional outcome, as documented by Teller visual acuity and formal occupational therapy testing, and anatomic outcome, as documented by Retcam photography and fluorescein angiography. Complete regression of extraretinal fibrovascular proliferation was observed 1 month after laser treatment. No retinal detachment had occurred to date at 24 months. Teller visual acuity at 23 months of life was 20/100 in both eyes. The patient's vision and developmental milestones were age appropriate. Pre-term genetic diagnosis with immediate laser treatment after birth may preserve vision in individuals affected with Norrie's disease. Copyright © 2010 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

A characteristic phenotypic retinal appearance in Norrie disease.

PubMed

Drenser, Kimberly A; Fecko, Alice; Dailey, Wendy; Trese, Michael T

2007-02-01

To describe a striking retinal finding that the authors have only seen in Norrie disease eyes and to determine if a particular genotype corresponds to this dramatic presentation. This is a retrospective, interventional case report of four patients seen in the clinic over a 1-year period. All patients had analysis of the Norrie gene by direct sequencing. All patients presented with a similar retinal appearance of dense stalk tissue, globular dystrophic retina, and peripheral avascular retina with pigmentary changes. Each patient was found to have a mutation in the Norrie gene affecting a cystine residue in the cystine knot domain. The mutations are predicted to disrupt the structure of the protein product, norrin, which is required for activation of the Wnt receptor:beta-catenin pathway. No other vitreoretinopathy that the authors have seen demonstrates this characteristic retinal presentation of severe retinal dysplasia. All four patients were found to have mutations in the Norrie gene which alter the cystine knot motif. Mutations affecting this domain appear to have devastating effects on retinal development and indicate phenotype correlates with mutations affecting the cystine knot domain.

[A literature review of Norrie disease].

PubMed

Ohba, N; Isashiki, Y

1996-02-01

Norrie disease is a rare genetic disorder characterized by bilateral congenital blindness. The salient clinical feature early in life is a dense, white, vascularized mass behind each lens due to maldeveloped retina. Cataracts and corneal opacities are developed in young childhood, followed by bulbar atrophies. Histopathologic examination suggests primary vitreoretinal dysplasia because of developmental arrest of the retina in the middle embryonic stage. Occasional patients show psychomotor retardation or progressive hearing loss as part of a multisystem disorder. The disease is transmitted by an X-linked recessive form of inheritance, with sons of female carriers having a 50% risk for expressing the disease. In recent years, a candidate gene for Norrie disease has been isolated and characterized, which encompasses 27 kilobases and consists of three exons interspersed by two introns. Microdeletions and a variety of point mutations in the disease gene were identified in Norrie patients, although the genotype-phenotype correlation remains to be defined, and molecular diagnosis is now available for Norrie disease. The encoded protein has homology to a protein domain involving mucins and TGF beta, which may play an essential role in targeting of retinal/neural connections.

Prenatal Diagnosis of a Case of Norrie Disease with Late Development of Bilateral Ocular Malformation.

PubMed

Wu, Li Hong; Chen, Li-Hong; Xie, Hongning; Xie, Ying-Jun

2017-06-01

We report a case of Norrie disease, diagnosed by prenatal ultrasound, confirmed by Sanger sequencing of the DNP gene from the aborted fetal cord blood and histologically. Prenatal ultrasound revealed no abnormality in either eye at 22 +1 and 31 +4 gestational weeks, but at 36 +5 gestational weeks both eyes had massive vitreous cavity opacities with complete retinal detachment. Norrie disease was initially suspected because of an older male sibling with the disease. To our knowledge, prenatal ultrasound diagnosis of Norrie disease has been previously described only one case in 1993 in a 34-week-old fetus. The normal eye development until after 31 + 4 gestational weeks provides insight into the first manifestation and then the rapid progression of the eye disease.

Bilateral persistent fetal vasculature due to a mutation in the Norrie disease protein gene

PubMed Central

Payabvash, Seyedmehdi; Anderson, Jill S

2015-01-01

We report a case of a 7-week-old boy with bilateral leukocoria and asymmetric microphthalmia who was found to have Norrie disease. Symmetrically hyperdense globes with no evidence of calcification were seen on CT scan. The MRI showed bilateral retinal hemorrhages resulting in conical vitreous chambers—narrow at the optic disc and widened toward the lens—characteristic of persistent fetal vasculature. Genetic evaluation revealed a previously undescribed mutation in the Norrie disease protein gene. PMID:26459204

Bilateral persistent fetal vasculature due to a mutation in the Norrie disease protein gene.

PubMed

Payabvash, Seyedmehdi; Anderson, Jill S; Nascene, David R

2015-12-01

We report a case of a 7-week-old boy with bilateral leukocoria and asymmetric microphthalmia who was found to have Norrie disease. Symmetrically hyperdense globes with no evidence of calcification were seen on CT scan. The MRI showed bilateral retinal hemorrhages resulting in conical vitreous chambers-narrow at the optic disc and widened toward the lens-characteristic of persistent fetal vasculature. Genetic evaluation revealed a previously undescribed mutation in the Norrie disease protein gene. © The Author(s) 2015.

Evaluation of Acid Ceramidase Overexpression-Induced Activation of the Oncogenic Akt Pathway in Prostate Cancer

DTIC Science & Technology

2014-01-01

Marrison, S.T., Norris , J.S., and Liu, X. 2013. Acid Ceramidase Promotes Nuclear Export of PTEN through Sphingosine 1-Phosphate Mediated Akt...cancer. Genes Chromo- somes Cancer 2000; 29: 137–146. 2 Norris JS, Bielawska A, Day T, El-Zawahri A, Elojeimy S, Hannun Y et al. Combined therapeutic use...Joseph C. Cheng, Ping Lu, S. Tucker Marrison, James S. Norris , Xiang Liu Department of Microbiology and Immunology, Medical University of South Carolina

Norrie disease in a family with a manifesting female carrier.

PubMed

Sims, K B; Irvine, A R; Good, W V

1997-04-01

To show that Norrie disease can occur in a girl and to describe her ophthalmologic and genetic features. Amplification of DNA polymerase chain reaction and sequencing of asymmetric polymerase chain reaction for exon 3 were performed on the blood specimen obtained from a girl born with bilateral retinal detachments. A female child with bilateral retinal detachment who had 2 uncles in whom Norrie disease had already been diagnosed. The child had a mutation in the third exon (T776-->A; Ile 123-->Asn) identical to the mutation found in her uncles. Norrie disease can occur in girls. The most likely explanation is nonrandom or unfavorable X inactivation, although timing of development of the peripheral retina and its blood supply could render it vulnerable to effects of the mutant allele at a critical developmental phase.

40 CFR 122.22 - Signatories to permit applications and reports (applicable to State programs, see § 123.25).

Code of Federal Regulations, 2014 CFR

2014-07-01

... initiating and directing other comprehensive measures to assure long term environmental compliance with....), Clean Air Act (42 U.S.C. 7401 et seq.), Resource Conservation and Recovery Act (42 U.S.C. 6901 et seq...

Source and fate of inorganic solutes in the Gibbon River, Yellowstone National Park, Wyoming, USA: I. Low-flow discharge and major solute chemistry

USGS Publications Warehouse

McCleskey, R. Blaine; Nordstrom, D. Kirk; Susong, David D.; Ball, James W.; Holloway, JoAnn M.

2010-01-01

The Gibbon River in Yellowstone National Park (YNP) is an important natural resource and habitat for fisheries and wildlife. However, the Gibbon River differs from most other mountain rivers because its chemistry is affected by several geothermal sources including Norris Geyser Basin, Chocolate Pots, Gibbon Geyser Basin, Beryl Spring, and Terrace Spring. Norris Geyser Basin is one of the most dynamic geothermal areas in YNP, and the water discharging from Norris is much more acidic (pH 3) than other geothermal basins in the upper-Madison drainage (Gibbon and Firehole Rivers). Water samples and discharge data were obtained from the Gibbon River and its major tributaries near Norris Geyser Basin under the low-flow conditions of September 2006. Surface inflows from Norris Geyser Basin were sampled to identify point sources and to quantify solute loading to the Gibbon River. The source and fate of the major solutes (Ca, Mg, Na, K, SiO2, Cl, F, HCO3, SO4, NO3, and NH4) in the Gibbon River were determined in this study and these results may provide an important link in understanding the health of the ecosystem and the behavior of many trace solutes. Norris Geyser Basin is the primary source of Na, K, Cl, SO4, and N loads (35–58%) in the Gibbon River. The largest source of HCO3 and F is in the lower Gibbon River reach. Most of the Ca and Mg originate in the Gibbon River upstream from Norris Geyser Basin. All the major solutes behave conservatively except for NH4, which decreased substantially downstream from Gibbon Geyser Basin, and SiO2, small amounts of which precipitated on mixing of thermal drainage with the river. As much as 9–14% of the river discharge at the gage is from thermal flows during this period.

[Analysis of the NDP gene in a Chinese family with X-linked recessive Norrie disease].

PubMed

Mei, Libin; Huang, Yanru; Pan, Qian; Liang, Desheng; Wu, Lingqian

2015-05-01

The purpose of the current research was to investigate the NDP (Norrie disease protein) gene in one Chinese family with Norrie disease (ND) and to characterize the related clinical features. Clinical data of the proband and his family members were collected. Complete ophthalmic examinations were carried out on the proband. Genomic DNA was extracted from peripheral blood leukocytes of 35 family members. Molecular analysis of the NDP gene was performed by polymerase chain reaction and direct sequencing of all exons and flanking regions. A hemizygous NDP missense mutation c.362G > A (p.Arg121Gln) in exon 3 was identified in the affected members, but not in any of the unaffected family individuals. The missense mutation c.362G > A in NDP is responsible for the Norrie disease in this family. This discovery will help provide the family members with accurate and reliable genetic counseling and prenatal diagnosis.

Norrie disease: first mutation report and prenatal diagnosis in an Indian family.

PubMed

Ghosh, Manju; Sharma, Shipra; Shastri, Shivaram; Arora, Sadhna; Shukla, Rashmi; Gupta, Neerja; Deka, Deepika; Kabra, Madhulika

2012-11-01

Norrie Disease (ND) is a rare X-linked recessive disorder characterised by congenital blindness due to severe retinal dysgenesis. Hearing loss and intellectual disability is present in 30-50 % cases. ND is caused by mutations in the NDP gene, located at Xp11.3. The authors describe mutation analysis of a proband with ND and subsequently prenatal diagnosis. Sequence analysis of the NDP gene revealed a hemizygous missense mutation arginine to serine in codon 41 (p.Arg41Ser) in the affected child. Mother was carrier for the mutation. In a subsequent di-chorionic di-amniotic pregnancy, the authors performed prenatal diagnosis by mutation analysis on chorionic villi sample at 11 wk of gestation. The fetuses were unaffected. This is a first mutation report and prenatal diagnosis of a familial case of Norrie disease from India. The importance of genetic testing of Norrie disease for confirmation, carrier testing, prenatal diagnosis and genetic counseling is emphasized.

Database of the Geology and Thermal Activity of Norris Geyser Basin, Yellowstone National Park

USGS Publications Warehouse

Flynn, Kathryn; Graham Wall, Brita; White, Donald E.; Hutchinson, Roderick A.; Keith, Terry E.C.; Clor, Laura; Robinson, Joel E.

2008-01-01

This dataset contains contacts, geologic units and map boundaries from Plate 1 of USGS Professional Paper 1456, 'The Geology and Remarkable Thermal Activity of Norris Geyser Basin, Yellowstone National Park, Wyoming.' The features are contained in the Annotation, basins_poly, contours, geology_arc, geology_poly, point_features, and stream_arc feature classes as well as a table of geologic units and their descriptions. This dataset was constructed to produce a digital geologic map as a basis for studying hydrothermal processes in Norris Geyser Basin. The original map does not contain registration tic marks. To create the geodatabase, the original scanned map was georegistered to USGS aerial photographs of the Norris Junction quadrangle collected in 1994. Manmade objects, i.e. roads, parking lots, and the visitor center, along with stream junctions and other hydrographic features, were used for registration.

A multitracer approach for characterizing interactions between shallow groundwater and the hydrothermal system in the Norris Geyser Basin area, Yellowstone National Park

USGS Publications Warehouse

Gardner, W.P.; Susong, D.D.; Solomon, D.K.; Heasler, H.P.

2011-01-01

Multiple environmental tracers are used to investigate age distribution, evolution, and mixing in local- to regional-scale groundwater circulation around the Norris Geyser Basin area in Yellowstone National Park. Springs ranging in temperature from 3??C to 90??C in the Norris Geyser Basin area were sampled for stable isotopes of hydrogen and oxygen, major and minor element chemistry, dissolved chlorofluorocarbons, and tritium. Groundwater near Norris Geyser Basin is comprised of two distinct systems: a shallow, cool water system and a deep, high-temperature hydrothermal system. These two end-member systems mix to create springs with intermediate temperature and composition. Using multiple tracers from a large number of springs, it is possible constrain the distribution of possible flow paths and refine conceptual models of groundwater circulation in and around a large, complex hydrothermal system. Copyright 2011 by the American Geophysical Union.

Norrie disease as part of a complex syndrome explained by a submicroscopic deletion of the X chromosome.

PubMed

Bleeker-Wagemakers, E M; Zweije-Hofman, I; Gal, A

1988-11-01

A 15-year-old male patient with the typical ocular symptoms of Norrie disease is described. Additionally, he presents severe mental retardation, growth disturbances, hypogonadism, and increased susceptibility to infections. This complex syndrome is apparently segregating through three generations: four other male relatives of the patient were blind from birth and died from recurrent infections between the ages of three to 15 months. The DNA sequence of the DXS7 locus (L1.28 probe), known to be closely linked to the Norrie gene, was not found in the patient's DNA. This result suggests that the more complex clinical picture seen is the result of a deletion of the X chromosome spanning DXS7, the Norrie gene, and several neighbouring loci. A detailed clinical description of the patient is given and compared to that of similar cases.

Localization of the Norrie disease gene mRNA by in situ hybridization.

PubMed

Hartzer, M K; Cheng, M; Liu, X; Shastry, B S

1999-07-15

Norrie disease is a rare X-linked recessive neurodevelopmental disorder. The affected males manifest congenital blindness, which is often associated with hearing loss, mental retardation and psychiatric problems. Genetic linkage studies have localized the gene to the short arm of the X-chromosome and the gene has been isolated recently. The encoded protein is a member of the superfamily of growth factors containing a cystine knot motif and may be involved in cell adhesion and neurodevelopment. Molecular genetic analysis revealed a large number of missense, nonsense, deletion, and splice-site mutations among Norrie patients. In order to further determine the role of the Norrie disease gene, we studied the distribution pattern of its mRNA in the retina and in brain by in situ hybridization. The results show abundant hybridization signals in outer nuclear, inner nuclear, and ganglion cell layers of the retina in all three species (mice, rabbit, and human) examined. There was no significant expression in the vitreous body, lens, and rod outer segment. High expression levels were also observed in the cerebellar granular layer, hippocampus, olfactory bulb, cortex, and epithelium of the rabbit brain. These data suggest that the Norrie disease gene could play a critical role in the differentiation or maintenance of the differentiated state of the retina.

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David Norris with Ford Focus electric cars

NASA Image and Video Library

2017-09-29

David Norris, Marshall transportation specialist, stands alongside two new, fully electric cars capable of traveling approximately 115 miles on a 5 1/2-hour charge using Marshall's 240-volt charging station. The electric cars join five "green" vehicles in use at Marshall since spring 2016.

Fetal loss in homozygous mutant Norrie disease mice: a new role of Norrin in reproduction.

PubMed

Luhmann, Ulrich F O; Meunier, Dominique; Shi, Wei; Lüttges, Angela; Pfarrer, Christiane; Fundele, Reinald; Berger, Wolfgang

2005-08-01

Mutations in the Norrie disease pseudoglioma gene (NDP) are known to cause X-linked recessive Norrie disease. In addition, NDP mutations have been found in other vasoproliferative retinopathies such as familial exudative vitreoretinopathy, retinopathy of prematurity, and Coats disease, suggesting a role for Norrin in vascular development. Here we report that female mice homozygous for the Norrie disease pseudoglioma homolog (Ndph) knockout allele exhibit almost complete infertility, while heterozygous females and hemizygous males are fertile. Histological examinations and RNA in situ hybridization analyses revealed defects in vascular development and decidualization in pregnant Ndph-/- females from embryonic day 7 (E7) onwards, resulting in embryonic loss. Using RT-PCR analyses we also demonstrate, for the first time, the expression of Ndph in mouse uteri and deciduae as well as the expression of NDP in human placenta. Taken together, these data provide strong evidence for Norrin playing an important role in female reproductive tissues. Copyright 2005 Wiley-Liss, Inc

A fetus with an X;1 balanced reciprocal translocation and eye disease.

PubMed Central

Seller, M J; Pal, K; Horsley, S; Davies, A F; Berry, A C; Meredith, R; McCartney, A C

1995-01-01

A 19 week female fetus is described with a de novo X;1 reciprocal balanced translocation, with the breakpoint on the X chromosome at Xp11.4, and eye pathology consistent with the early stages of Norrie disease. The fetus seems to be an example of a female manifesting an X linked recessive disease, and it was shown that the normal X chromosome was completely inactivated in all cells examined. Norrie disease has been mapped to Xp11.3, and fluorescence in situ hybridisation studies showed that the Norrie disease gene had not obviously been disrupted. Mutation screening by SSCP analysis showed no aberrant fragments of the coding region of the gene. Several eye disease genes map to the same region of the X chromosome, but are excluded on grounds of pathology. One possibility is that this fetus has a Norrie-like eye disease caused by the mutation of another gene located at Xp11.4. If this is so, there are implications for prenatal diagnosis. Images PMID:7562972

Ornithine aminotransferase (OAT): recombination between an X-linked OAT sequence (7.5 kb) and the Norrie disease locus.

PubMed

Ngo, J T; Bateman, J B; Spence, M A; Cortessis, V; Sparkes, R S; Kivlin, J D; Mohandas, T; Inana, G

1990-01-01

A human ornithine aminotransferase (OAT) locus has been mapped to the Xp11.2, as has the Norrie disease locus. We used a cDNA probe to investigate a 3-generation UCLA family with Norrie disease; a 4.2-kb RFLP was detected and a maximum lod score of 0.602 at zero recombination fraction was calculated. We used the same probe to study a second multigeneration family with Norrie disease from Utah. A different RFLP of 7.5 kb in size was identified and a recombinational event between the OAT locus represented by this RFLP and the disease loci was observed. Linkage analysis of these two loci in this family revealed a maximum load score of 1.88 at a recombination fraction of 0.10. Although both families have affected members with the same disease, the lod scores are reported separately because the 4.2- and 7.5-kb RFLPs may represent two different loci for the X-linked OAT.

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Recombinational event between Norrie disease and DXS7 loci.

PubMed

Ngo, J T; Spence, M A; Cortessis, V; Sparkes, R S; Bateman, J B

1988-07-01

We have identified a family affected with X-linked recessive Norrie disease, in which a recombinational event occurred between the disease locus and the DXS7 locus identified by the probe L1.28. The addition of our family brings the total of published informative families to seven, with a maximum lod score of 7.58 at a recombination frequency of 0.038 +/- 0.036. This finding indicates that the L1.28 probe is useful but may not be completely reliable for prenatal diagnosis and that the gene for Norrie disease is not within the DNA sequence identified by the L1.28 probe.

In Search of Activist Pedagogies in SMTE

ERIC Educational Resources Information Center

Alsop, Steve; Bencze, Larry

2012-01-01

David Burns and Stephen Norris's (2012) article entitled "Activist Environmental Education and Moral Philosophy" offers a thought-provoking response to the CJSMTE special edition. The authors would like to thank these authors for their supportive and philosophically adroit arguments. Burns and Norris provide an opportunity to continue…

Ectopic norrin induces growth of ocular capillaries and restores normal retinal angiogenesis in Norrie disease mutant mice.

PubMed

Ohlmann, Andreas; Scholz, Michael; Goldwich, Andreas; Chauhan, Bharesh K; Hudl, Kristiane; Ohlmann, Anne V; Zrenner, Eberhart; Berger, Wolfgang; Cvekl, Ales; Seeliger, Mathias W; Tamm, Ernst R

2005-02-16

Norrie disease is an X-linked retinal dysplasia that presents with congenital blindness, sensorineural deafness, and mental retardation. Norrin, the protein product of the Norrie disease gene (NDP), is a secreted protein of unknown biochemical function. Norrie disease (Ndp(y/-)) mutant mice that are deficient in norrin develop blindness, show a distinct failure in retinal angiogenesis, and completely lack the deep capillary layers of the retina. We show here that the transgenic expression of ectopic norrin under control of a lens-specific promoter restores the formation of a normal retinal vascular network in Ndp(y/-) mutant mice. The improvement in structure correlates with restoration of neuronal function in the retina. In addition, lenses of transgenic mice with ectopic expression of norrin show significantly more capillaries in the hyaloid vasculature that surrounds the lens during development. In vitro, lenses of transgenic mice in coculture with microvascular endothelial cells induce proliferation of the cells. Transgenic mice with ectopic expression of norrin show more bromodeoxyuridine-labeled retinal progenitor cells at embryonic day 14.5 and thicker retinas at postnatal life than wild-type littermates, indicating a putative direct neurotrophic effect of norrin. These data provide direct evidence that norrin induces growth of ocular capillaries and that pharmacologic modulation of norrin might be used for treatment of the vascular abnormalities associated with Norrie disease or other vascular disorders of the retina.

Co-segregation of Norrie disease and idiopathic pulmonary hypertension in a family with a microdeletion of the NDP region at Xp11.3-p11.4.

PubMed

Staropoli, John F; Xin, Winnie; Sims, Katherine B

2010-11-01

Norrie disease is a rare X-linked congenital retinal vasculopathy that may be accompanied by sensorineural deafness, mental retardation, and other neurological deficits. Here we present a family in which Norrie disease co-segregated with either early-onset idiopathic pulmonary hypertension or sudden death preceded by a period of progressive dyspnea. Neither Norrie disease, nor its atypical variants described to date, have been associated with this extended clinical phenotype. Molecular analysis of the Norrie disease gene (NDP) and adjacent loci was performed by multiplex ligation-dependent probe amplification and comparative genomic hybridisation. Affected males in this family showed an inherited hemizygous deletion restricted to NDP and two immediately telomeric genes, monoamine oxidase-B (MAO-B) and monoamine oxidase-A (MAO-A), which encode closely related enzymes that metabolize biogenic amines including serotonin, dopamine, and norepinephrine. Sequencing of the deletion junction showed an unusual pattern in which a region of microhomology flanked intervening genomic sequence. Because abnormalities of biogenic amines, particularly serotonin, have been implicated in the pathophysiology of pulmonary hypertension, we propose that presumed MAO deficiency in these patients may represent a novel risk factor for pulmonary hypertension, particularly forms with very early onset. Fine-mapping of other microdeletions at this locus may provide insights into additional mechanisms for nonrecurrent genomic rearrangements at this and other chromosomal loci.

Further linkage data on Norrie disease.

PubMed

Kivlin, J D; Sanborn, G E; Wright, E; Cannon, L; Carey, J

1987-03-01

We obtained a LOD score of +1.61 using DNA marker L1.28 in 5 generations of a family with Norrie disease, raising the total LOD score to +5.42. There have been no recombinations between the 2 loci in any family to date, making the marker useful for genetic counseling.

NREL Rewards Teenaged Renewable Energy Expert at International Competition

Science.gov Websites

Rewards Teenaged Renewable Energy Expert at International Competition For more information contact Midwest Research Institute selected Josephine Norris from Detroit, Mich. to receive a special renewable energy award at the International Science and Engineering Fair (ISEF) in Fort Worth, Texas. Norris will

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Results of weekly chemical and isotopic monitoring of selected springs in Norris Geyser Basin, Yellowstone National Park during June-September, 1995

USGS Publications Warehouse

Fournier, R.O.; Weltman, U.; Counce, D.; White, L.D.; Janik, C.J.

2002-01-01

Each year at Norris Geyser Basin, generally in August or September, a widespread hydrothermal 'disturbance' occurs that is characterized by simultaneous changes in the discharge characteristics of many springs, particularly in the Back Basin. During the summer season of 1995, water samples from eight widely distributed hot springs and geysers at Norris were collected each week and analyzed to determine whether chemical and isotopic changes also occurred in the thermal waters at the time of the disturbance. In addition, Beryl Spring in Gibbon Canyon, 5.8 km southwest of Norris Geyser Basin, was included in the monitoring program. Waters discharged by four of the monitored hot springs and geysers appear to issue from relatively deep reservoirs where temperatures are at least 270 C and possibly higher than 300 C. At the time of, and for several days after, the onset of the 1995 disturbance, the normally neutral-chloride waters discharged by these four features all picked up an acid-sulfate component and became isotopically heavier. The acid-sulfate component appears to be similar in composition to some waters discharged in 100 Spring Plain that issue from subsurface regions where temperatures are in the range 170-210 C. However, the two monitored springs that discharge acid-chloride-sulfate waters in the 100 Spring Plain region did not show any significant chemical or isotopic response to the annual disturbance. Beryl Spring, and two neutral-chloride hot springs at Norris that appear to draw their water from reservoirs where temperatures are 250 C or less, also did not show any significant chemical or isotopic response to the annual disturbance. After the start of the annual disturbance, chloride concentrations in water sampled from Double Bulger Geyser in the Back Basin increased from about 800 ppm to about 1500 ppm, nearly twice as high as any previously reported chloride concentration in a thermal water at Yellowstone. The isotopic composition of that water precludes an origin of the high chloride by evaporation at atmospheric pressure. One way to account for the unique chemical and isotopic composition of this highly concentrated wateris by recirculation of water that had gone through one cycle of adiabatic cooling during upflow (decompressional boiling) back down into the hydrothermal system, where it is reheated to greater than 220 C. This previously boiled water then undergoes additional cycles of decompressional boiling during subsequent upflow. Another way the unique chemical and isotopic composition of Double Bulger water might evolve is by excess boiling in the formation that results from a decrease in fluid pressure within the channels of upflow. The annual disturbance at Norris Geyser Basin generally appears to be triggered by a cyclic up and down movement of the boilingpoint curve within the hydrothermal system in response to changes in the potentiometric surface of the cold water that is adjacent to, and interconnected with, that hydrothermal system. Annual disturbance phenomena that are easily recognized at Norris Geyser Basin may not be easily recognized elsewhere in Yellowstone National Park because (1) the neutral-chloride waters at Norris ascend directly from higher-temperature and higherpressure reservoirs (270 to >300 C at Norris compared to 180-215C at Upper and Lower Geyser Basins) that are capable of producing massive amounts of high-pressure steam, and (2) the clay that makes hot spring and geyser waters become turbid at Norris, heralding the start of the disturbance, comes from acid altered rocks that are widely distributed at intermediate depths at Norris, and that are rare in other geyser basins.

Voltage-dependent ion channels in the mouse RPE: comparison with Norrie disease mice.

PubMed

Wollmann, Guido; Lenzner, Steffen; Berger, Wolfgang; Rosenthal, Rita; Karl, Mike O; Strauss, Olaf

2006-03-01

We studied electrophysiological properties of cultured retinal pigment epithelial (RPE) cells from mouse and a mouse model for Norrie disease. Wild-type RPE cells revealed the expression of ion channels known from other species: delayed-rectifier K(+) channels composed of Kv1.3 subunits, inward rectifier K(+) channels, Ca(V)1.3 L-type Ca(2+) channels and outwardly rectifying Cl(-) channels. Expression pattern and the ion channel characteristics current density, blocker sensitivity, kinetics and voltage-dependence were compared in cells from wild-type and Norrie mice. Although no significant differences were observed, our study provides a base for future studies on ion channel function and dysfunction in transgenic mouse models.

The Future Is in Your Hand

ERIC Educational Resources Information Center

Traylor, Scott

2009-01-01

This article presents an interview with Cathleen Norris and Elliot Soloway, both pioneering educators who are defining the future of technology and learning. Norris is a professor in the Department of Technology and Cognition at the University of North Texas. She is also the past president of ISTE and the past president of NECA, the organizing…

Norrie disease. Diagnosis of a simplex case by DNA analysis.

PubMed

Chynn, E W; Walton, D S; Hahn, L B; Dryja, T P

1996-09-01

Norrie disease is a rare, X-linked recessive disorder characterized by congenital blindness due to malformed retinas. We describe a simplex patient who had leukokoria and whose clinical diagnosis was confirmed only after molecular genetics analysis. DNA analysis was also used to determine the carrier status of relatives of the proband.

On Consumerism, Collective Action, and Whether Art Teaches Anything

ERIC Educational Resources Information Center

Ruitenberg, Claudia W.

2014-01-01

In this review essay, Claudia Ruitenberg discusses Trevor Norris's "Consuming Schools," René Arcilla's "Mediumism," and Martha Nussbaum's "Not for Profit." While the primary focus of each book is different--with Norris concentrating on the pressures of consumerism and commercialism on K-12 schooling,…

Cochlear Implants and Psychiatric Assessments: a Norrie Disease Case Report.

PubMed

Jacques, Denis; Dubois, Thomas; Zdanowicz, Nicolas; Gilain, Chantal; Garin, Pierre

2017-09-01

It is important to perform psychiatric assessments of adult patients who are candidates for cochlear implants both to screen them for psychiatric disorders and to assess their understanding and compliance with the procedure. Deafness is a factor of difficulty for conducting in-depth psychiatric interviews, but concomitant blindness may make it impossible. After a description of Norrie disease, a rare disease in which blindness and deafness may occur together, we propose a case report of a patient suffering from the disease and who consulted in view of a cochlear implant. Early information on cochlear implants appears to be necessary before total deafness occurs in patients suffering from Norrie disease. An inventory of digital communication tools that can be used by the patient is also highly valuable. Research should be supported for a more systematic use of psychiatric assessments prior to cochlear implants. In the special case of Norrie disease, we recommend early screening for mental retardation and related psychotic disorders and, depending on the patient's level of understanding, preventive information on the benefits and limits of cochlear implants before total deafness occurs.

Familial cases of Norrie disease detected by copy number analysis.

PubMed

Arai, Eisuke; Fujimaki, Takuro; Yanagawa, Ai; Fujiki, Keiko; Yokoyama, Toshiyuki; Okumura, Akihisa; Shimizu, Toshiaki; Murakami, Akira

2014-09-01

Norrie disease (ND, MIM#310600) is an X-linked disorder characterized by severe vitreoretinal dysplasia at birth. We report the results of causative NDP gene analysis in three male siblings with Norrie disease and describe the associated phenotypes. Three brothers with suspected Norrie disease and their mother presented for clinical examination. After obtaining informed consent, DNA was extracted from the peripheral blood of the proband, one of his brothers and his unaffected mother. Exons 1-3 of the NDP gene were amplified by polymerase chain reaction (PCR), and direct sequencing was performed. Multiplex ligation-dependent probe amplification (MLPA) was also performed to search for copy number variants in the NDP gene. The clinical findings of the three brothers included no light perception, corneal opacity, shallow anterior chamber, leukocoria, total retinal detachment and mental retardation. Exon 2 of the NDP gene was not amplified in the proband and one brother, even when the PCR primers for exon 2 were changed, whereas the other two exons showed no mutations by direct sequencing. MLPA analysis showed deletion of exon 2 of the NDP gene in the proband and one brother, while there was only one copy of exon 2 in the mother. Norrie disease was diagnosed in three patients from a Japanese family by clinical examination and was confirmed by genetic analysis. To localize the defect, confirmation of copy number variation by the MLPA method was useful in the present study.

Norrie disease and exudative vitreoretinopathy in families with affected female carriers.

PubMed

Shastry, B S; Hiraoka, M; Trese, D C; Trese, M T

1999-01-01

Norrie disease (ND) is a rare X-linked recessive disorder characterized by congenital blindness, which is often associated with sensorineural hearing loss and mental retardation. X-linked familial exudative vitreoretinopathy (FEVR) is a hereditary disorder characterized by an abnormality of the peripheral retina and is not associated with systemic diseases. X-linked recessive disorders generally do not affect females. Here we show that female carriers can be associated with manifestation of an X-linked disorder. A four-generation family with an affected female, and a history of congenital blindness and hearing loss, was identified through the pro-band. A second family, with a full-term female infant, was evaluated through ophthalmic examinations and found to exhibit ocular features, such as retinal folds, retinal detachment and peripheral exudates. Peripheral blood specimens were collected from several affected and unaffected family members. DNA was extracted and analyzed by single-strand conformation polymorphism (SSCP) following polymerase chain reaction (PCR) amplification of the exons of the Norrie disease gene. The amplified products were sequenced by the dideoxy chain termination method. In an X-linked four-generation family, a novel missense (A118D) mutation in the third exon of the Norrie disease gene, was identified. The mutation was transmitted through three generations and cosegregated with the disease. The affected maternal grandmother and the unaffected mother carried the same mutation in one of their alleles. In an unrelated sporadic family, a heterozygous missense mutation (C96Y) was identified in the third exon of the Norrie disease gene in an affected individual. Analysis of exon-1 and 2 of the Norrie disease gene did not reveal any additional sequence alterations in these families. The mutations were not detected in the unaffected family members and the 116 normal unrelated controls, suggesting that they are likely to be the pathogenic mutations. The results further strengthen the proposal that X-linked disorders can occur in female carriers, due likely to an unfavorable X-inactivation.

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4 Wheel City

ERIC Educational Resources Information Center

Henderson, Nancy

2009-01-01

Namel Norris and Ricardo Velasquez are two young men who had never met, yet they shared much in common. Both sustained spinal cord injuries and were in wheelchairs. Both felt shunned by the people they used to hang out with. Before his accident, Norris had been an avid basketball player and member of a rap group trying to make it in the music…

Diving in Head First: Finding the Volume of Norris lake

ERIC Educational Resources Information Center

Foster, Drew W.

2008-01-01

This article allows students to apply their knowledge and experience of area and volume to find the volume of Norris Lake, a large reservoir lake in Tennessee. Students have the opportunity to demonstrate their skills in using maps and scales as well as to incorporate the use of technology in developing the solution. This project satisfied the…

Keratotorus in Norrie disease.

PubMed

Lang, G E; Rott, H D; Naumann, G O

1991-08-01

We report on a 46-year old white male with Norrie disease. In the right eye he had a keratotorus with non vascularized corneal scars and mature cataract. After penetrating keratoplasty a pseudoglioma with irreversible total retinal detachment was found. The left eye had phthisis bulbi with corneal pannus and band keratopathy, shallow anterior chamber, posterior synechia and mature cataract.

Coats' disease, Turner syndrome, and von Willebrand disease in a patient with Wildtype Norrie disease pseudoglioma.

PubMed

Desai, Rajen U; Saffra, Norman A; Krishna, Rati P; Rosenberg, Steven E

2011-01-01

The authors describe a girl diagnosed as having Coats' disease, Turner syndrome (45X karyotype), and type 1 von Willebrand disease. She tested negative for the Norrie disease pseudoglioma (NDP) gene located on the X-chromosome, which has been suspected of contributing to Coats' disease. Copyright 2010, SLACK Incorporated.

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Role of the Norrie disease pseudoglioma gene in sprouting angiogenesis during development of the retinal vasculature.

PubMed

Luhmann, Ulrich F O; Lin, Jihong; Acar, Niyazi; Lammel, Stefanie; Feil, Silke; Grimm, Christian; Seeliger, Mathias W; Hammes, Hans-Peter; Berger, Wolfgang

2005-09-01

To characterize developmental defects and the time course of Norrie disease in retinal and hyaloid vasculature during retinal development and to identify underlying molecular angiogenic pathways that may be affected in Norrie disease, exudative vitreoretinopathy, retinopathy of prematurity, and Coats' disease. Norrie disease pseudoglioma homologue (Ndph)-knockout mice were studied during retinal development at early postnatal (p) stages (p5, p10, p15, and p21). Histologic techniques, quantitative RT-PCR, ELISA, and Western blot analyses provided molecular data, and scanning laser ophthalmoscopy (SLO) angiography and electroretinography (ERG) were used to obtain in vivo data. The data showed that regression of the hyaloid vasculature of Ndph-knockout mice occurred but was drastically delayed. The development of the superficial retinal vasculature was strongly delayed, whereas the deep retinal vasculature did not form because of the blockage of vessel outgrowth into the deep retinal layers. Subsequently, microaneurysm-like lesions formed. Several angiogenic factors were differentially transcribed during retinal development. Increased levels of hypoxia inducible factor-1alpha (HIF1alpha) and VEGFA, as well as a characteristic ERG pattern, confirmed hypoxic conditions in the inner retina of the Ndph-knockout mouse. These data provide evidence for a crucial role of Norrin in hyaloid vessel regression and in sprouting angiogenesis during retinal vascular development, especially in the development of the deep retinal capillary networks. They also suggest an early and a late phase of Norrie disease and may provide an explanation for similar phenotypic features of allelic retinal diseases in mice and patients as secondary consequences of pathologic hypoxia.

Disrupting the Able-Bodied Normativity of Shared Power in the Duoethnographic Process: A Critical, Disability Studies Lens

ERIC Educational Resources Information Center

Nusbaum, Emily A.; Sitter, Kathleen C.

2016-01-01

Duoethnography (DE) is a collaborative research method where two or more individuals explore similar and different meanings of a phenomenon, based on each of their life experiences (Norris, 2008). Created by Joe Norris and Rick Sawyer, the approach is informed by the narrative tradition of storytelling and builds on Pinar's concept of…

Is That What Bayesians Believe? Reply to Griffiths, Chater, Norris, and Pouget (2012)

ERIC Educational Resources Information Center

Bowers, Jeffrey S.; Davis, Colin J.

2012-01-01

Griffiths, Chater, Norris, and Pouget (2012) argue that we have misunderstood the Bayesian approach. In their view, it is rarely the case that researchers are making claims that performance in a given task is near optimal, and few, if any, researchers adopt the theoretical Bayesian perspective according to which the mind or brain is actually…

Norrie disease: linkage analysis using a 4.2-kb RFLP detected by a human ornithine aminotransferase cDNA probe.

PubMed

Ngo, J T; Bateman, J B; Cortessis, V; Sparkes, R S; Mohandas, T; Inana, G; Spence, M A

1989-05-01

Previous study has shown that the usual DNA marker for Norrie disease, the L1.28 probe which identifies the DXS7 locus, can recombine with the disease locus. In this study, we used a human ornithine aminotransferase (OAT) cDNA which detects OAT-related DNA sequences mapped to the same region on the X chromosome as that of the L1.28 probe to investigate the family with Norrie disease who exhibited the recombinational event. When genomic DNA from this family was digested with the PvuII restriction endonuclease, we found a restriction fragment length polymorphism (RFLP) of 4.2 kb in size. This fragment was absent in the affected males and cosegregated with the disease locus; we calculated a lod score of 0.602, at theta = 0.00. No deletion could be detected by chromosomal analysis or on Southern blots with other enzymes. These results suggest that one of the OAT-related sequences on the X chromosome may be in close proximity to the Norrie disease locus and represent the first report which indicates that the OAT cDNA may be useful for the identification of carrier status and/or prenatal diagnosis.

[Analysis of gene mutation in a Chinese family with Norrie disease].

PubMed

Zhang, Tian-xiao; Zhao, Xiu-li; Hua, Rui; Zhang, Jin-song; Zhang, Xue

2012-09-01

To detect the pathogenic mutation in a Chinese family with Norrie disease. Clinical diagnosis was based on familial history, clinical sign and B ultrasonic examination. Peripheral blood samples were obtained from all available members in a Chinese family with Norrie disease. Genomic DNA was extracted from lymphocytes by the standard SDS-proteinase K-phenol/chloroform method. Two coding exons and all intron-exon boundaries of the NDP gene were PCR amplified using three pairs of primers and subjected to automatic DNA sequence. The causative mutation was confirmed by restriction enzyme analysis and genotyping analysis in all members. Sequence analysis of NDP gene revealed a missense mutation c.220C > T (p.Arg74Cys) in the proband and his mother. Further mutation identification by restriction enzyme analysis and genotyping analysis showed that the proband was homozygote of this mutation. His mother and other four unaffected members (III3, IV4, III5 and II2) were carriers of this mutation. The mutant amino acid located in the C-terminal cystine knot-like domain, which was critical motif for the structure and function of NDP. A NDP missense mutation was identified in a Chinese family with Norrie disease.

Screening for NDP mutations in 44 unrelated patients with familial exudative vitreoretinopathy or Norrie disease.

PubMed

Yang, Huiqin; Li, Shiqiang; Xiao, Xueshan; Guo, Xiangming; Zhang, Qingjiong

2012-08-01

To screen mutations in the norrin (NDP) gene in 44 unrelated Chinese patients with familial exudative vitreoretinopathy (FEVR, 38 cases) or Norrie disease (6 cases) and to describe the associated phenotypes. Of the 44 patients, mutation in FZD4, LRP5, and TSPAN12 was excluded in 38 patients with FEVR in previous study. Sanger sequencing was used to analyze the 2 coding exons and their adjacent regions of NDP in the 44 patients. Clinical data were presented for patients with mutation. NDP variants in 5 of the 6 patients with Norrie disease were identified, including a novel missense mutation (c.164G>A, p.Cys55Phe) in one patient, two known missense mutations (c.122G>A, p.Arg41Lys; c.220C>T, p.Arg74Cys) in two patients, and a gross deletion encompassing the two coding exons in two patients. Of the 5 patients, 3 had a family history and 2 were singleton cases. No mutation in NDP was detected in the 38 patients with FEVR. NDP mutations are common cause of Norrie disease but might be rare cause for FEVR in Chinese.

Molecular modelling of the Norrie disease protein predicts a cystine knot growth factor tertiary structure.

PubMed

Meitinger, T; Meindl, A; Bork, P; Rost, B; Sander, C; Haasemann, M; Murken, J

1993-12-01

The X-lined gene for Norrie disease, which is characterized by blindness, deafness and mental retardation has been cloned recently. This gene has been thought to code for a putative extracellular factor; its predicted amino acid sequence is homologous to the C-terminal domain of diverse extracellular proteins. Sequence pattern searches and three-dimensional modelling now suggest that the Norrie disease protein (NDP) has a tertiary structure similar to that of transforming growth factor beta (TGF beta). Our model identifies NDP as a member of an emerging family of growth factors containing a cystine knot motif, with direct implications for the physiological role of NDP. The model also sheds light on sequence related domains such as the C-terminal domain of mucins and of von Willebrand factor.

ON NORRIS' THEORY FOR THE SHAPE OF THE MAMMALIAN ERYTHROCYTE

PubMed Central

Ponder, Eric

1934-01-01

This paper is concerned with an attempt to put Norris' theory for the shape of the mammalian erythrocyte into a quantitative form. The theory supposes that the biconcave form of the cell is brought about by an expansive force enlarging the surface, and is also supposed to apply to the formation of the myelin forms of lecithn. The attempt is not successful, and is published merely because it is suggestive. Various points regarding the shape of the cell, the curvature of its surface, and the kind of system to which Norris' theory might be supposed to apply, are discussed, and an empirical formula is given for the curve which bounds the cross-section of the cell. This empirical formula describes the shape almost to perfection. PMID:19872803

75 FR 22785 - Proposed Administrative Settlement Agreement Under Section 122 of the Comprehensive Environmental...

Federal Register 2010, 2011, 2012, 2013, 2014

2010-04-30

... Leaman Tank Lines, Inc. Superfund Site Located in Logan Township, Gloucester County, NJ AGENCY..., Inc. (the ``Settling Party'') pursuant to Section 122 of the Comprehensive Environmental Response, Compensation, and Liability Act (``CERCLA''), 42 U.S.C. 9622. The Settlement Agreement provides for Settling...

A mutation in the Norrie disease gene (NDP) associated with X-linked familial exudative vitreoretinopathy.

PubMed

Chen, Z Y; Battinelli, E M; Fielder, A; Bundey, S; Sims, K; Breakefield, X O; Craig, I W

1993-10-01

Familial exudative vitreoretinopathy (FEVR) is a hereditary disorder characterized by an abnormality of the peripheral retina. Both autosomal dominant (adFEVR) and X-linked (XLFEVR) forms have been described, but the biochemical defect(s) underlying the symptoms are unknown. Molecular analysis of the Norrie gene locus (NDP) in a four generation FEVR family (shown previously to exhibit linkage to the X-chromosome markers DXS228 and MAOA (Xp11.4-p11.3)) reveals a missense mutation in the highly conserved region of the NDP gene, which caused a neutral amino acid substitution (Leu124Phe), was detected in all of the affected males, but not in the unaffected family members, nor in normal controls. The observations suggest that phenotypes of both XLFEVR and Norrie disease can result from mutations in the same gene.

RBC Storage Effect on Coagulation, Microparticles and Microchimerism in Critically Ill Patients

DTIC Science & Technology

2014-01-01

Effect on Coagulation, Microparticles and Microchimerism in Critically Ill Patients.” PRINCIPAL INVESTIGATOR: Phillip Norris , MD CONTRACTING...NUMBER 6. AUTHOR(S) Philip J. Norris , MD 5d. PROJECT NUMBER Philip Spinella, MD 5e. TASK NUMBER Avani Shah, MPH 5f. WORK UNIT NUMBER 7...severity of multiple organ dysfunction syndrome , serious thrombotic events and nosocomial infections, and ICU and hospital length of stay

Repair of Total Tractional Retinal Detachment in Norrie Disease: Report of Technique and Successful Surgical Outcome.

PubMed

Todorich, Bozho; Thanos, Aristomenis; Yonekawa, Yoshihiro; Capone, Antonio

2017-03-01

Norrie disease is a rare, but devastating cause of pediatric retinal detachment, universally portending a poor visual prognosis. This paper describes successful surgical management of an infant with total retinal detachment associated with Norrie disease mutation. The infant was a full-term white male who presented with bilateral total funnel retinal detachments (RDs). He underwent genetic testing, which demonstrated single-point mutation 133 G>A transition in exon 2 of the NDP gene. The retinal detachment was managed with translimbal iridectomy, lensectomy, capsulectomy, and vitrectomy. Careful dissection of the retrolental membranes resulted in opening of the funnel. Single-stage surgery in this child's eye achieved re-attachment of the posterior pole with progressive reabsorption of subretinal fluid and cholesterol without the need for external drainage. Fluorescein angiography, performed at 2 months postoperatively, demonstrated perfusion of major vascular arcades, but with significant abnormalities and aneurysmal changes of higher-order vessels, suggestive of retinal and vascular dysplasia. The child has maintained brisk light perception vision. Early surgical intervention with careful dissection of tractional tissues can potentially result in good anatomic outcomes in some patients with Norrie disease-associated retinal detachment. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:260-262.]. Copyright 2017, SLACK Incorporated.

Retinal phenotype-genotype correlation of pediatric patients expressing mutations in the Norrie disease gene.

PubMed

Wu, Wei-Chi; Drenser, Kimberly; Trese, Michael; Capone, Antonio; Dailey, Wendy

2007-02-01

To correlate the ophthalmic findings of patients with pediatric vitreoretinopathies with mutations occurring in the Norrie disease gene (NDP). One hundred nine subjects with diverse pediatric vitreoretinopathies and 54 control subjects were enrolled in the study. Diagnoses were based on retinal findings at each patient's first examination. Samples of DNA from each patient underwent polymerase chain reaction amplification and direct sequencing of the NDP gene. Eleven male patients expressing mutations in the NDP gene were identified in the test group, whereas the controls demonstrated wild-type NDP. All patients diagnosed as having Norrie disease had mutations in the NDP gene. Four of the patients with Norrie disease had mutations involving a cysteine residue in the cysteine-knot motif. Four patients diagnosed as having familial exudative vitreoretinopathy were found to have noncysteine mutations. One patient with retinopathy of prematurity had a 14-base deletion in the 5' untranslated region (exon 1), and 1 patient with bilateral persistent fetal vasculature syndrome expressed a noncysteine mutation in the second exon. Mutations disrupting the cysteine-knot motif corresponded to severe retinal dysgenesis, whereas patients with noncysteine mutations had varying degrees of avascular peripheral retina, extraretinal vasculature, and subretinal exudate. Patients exhibiting severe retinal dysgenesis should be suspected of carrying a mutation that disrupts the cysteine-knot motif in the NDP gene.

Clinical, biochemical, and neuropsychiatric evaluation of a patient with a contiguous gene syndrome due to a microdeletion Xp11.3 including the Norrie disease locus and monoamine oxidase (MAOA and MAOB) genes.

PubMed

Collins, F A; Murphy, D L; Reiss, A L; Sims, K B; Lewis, J G; Freund, L; Karoum, F; Zhu, D; Maumenee, I H; Antonarakis, S E

1992-01-01

Norrie disease is a rare X-linked recessive disorder characterized by blindness from infancy. The gene for Norrie disease has been localized to Xp11.3. More recently, the genes for monoamine oxidase (MAOA, MAOB) have been mapped to the same region. This study evaluates the clinical, biochemical, and neuropsychiatric data in an affected male and 2 obligate heterozygote females from a single family with a submicroscopic deletion involving Norrie disease and MAO genes. The propositus was a profoundly retarded, blind male; he also had neurologic abnormalities including myoclonus and stereotopy-habit disorder. Both obligate carrier females had a normal IQ. The propositus' mother met diagnostic criteria for "chronic hypomania and schizotypal features." The propositus' MAO activity was undetectable and the female heterozygotes had reduced levels comparable to patients receiving MAO inhibiting antidepressants. MAO substrate and metabolite abnormalities were found in the propositus' plasma and CSF. This study indicates that subtle biochemical and possibly neuropsychiatric abnormalities may be detected in some heterozygotes with the microdeletion in Xp11.3 due to loss of the gene product for the MAO genes; this deletion can also explain some of the complex phenotype of this contiguous gene syndrome in the propositus.

75 FR 13110 - Proposed Waivers for the Comprehensive Centers Program and Funding of Continuation Grants

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2010-03-18

... schools to close achievement gaps in core content areas and raise student achievement in schools... 1116 of the ESEA. Eligible applicants for Comprehensive Center grants are research organizations... grantees from the account-closing provisions in 31 U.S.C. 1552(a), nor would they extend the availability...

US Space Superiority: It was Good While it Lasted; Exploring the Contesting and Congesting of the Space Domain

DTIC Science & Technology

2012-04-11

of the Graduation Requirements Advisors: Dr. John T. Ackerman Dr. Kathleen Mahoney -Norris Maxwell Air Force...like to thank my advisors, Dr. John Ackerman and Dr. Kathleen Mahoney -Norris, for creating and administering the Future Trends course with such...Security Act of 2011. 49 Newman , Andrew. The Truth About Oil. 50 Miner, Dan. Beyond Oil: Appendix B: Fuel Depletion and Peak Oil Guarantee Volatility

Utilization of gene mapping and candidate gene mutation screening for diagnosing clinically equivocal conditions: a Norrie disease case study.

PubMed

Chini, Vasiliki; Stambouli, Danai; Nedelea, Florina Mihaela; Filipescu, George Alexandru; Mina, Diana; Kambouris, Marios; El-Shantil, Hatem

2014-06-01

Prenatal diagnosis was requested for an undiagnosed eye disease showing X-linked inheritance in a family. No medical records existed for the affected family members. Mapping of the X chromosome and candidate gene mutation screening identified a c.C267A[p.F89L] mutation in NPD previously described as possibly causing Norrie disease. The detection of the c.C267A[p.F89L] variant in another unrelated family confirms the pathogenic nature of the mutation for the Norrie disease phenotype. Gene mapping, haplotype analysis, and candidate gene screening have been previously utilized in research applications but were applied here in a diagnostic setting due to the scarcity of available clinical information. The clinical diagnosis and mutation identification were critical for providing proper genetic counseling and prenatal diagnosis for this family.

A novel c.240_241insGG mutation in NDP gene in a family with Norrie disease.

PubMed

Andarva, Monavvar; Jamshidi, Javad; Ghaedi, Hamid; Daftarian, Narsis; Emamalizadeh, Babak; Alehabib, Elham; Taghavi, Shaghyegh; Pouriran, Ramin; Darvish, Hossein

2018-03-01

Norrie disease (ND) is a rare, X-linked recessive disorder with the main characteristic of early childhood blindness. The aim of the present study was to identify the genetic cause of the disease and the phenotypic characteristics of the patients in an Iranian family with four affected males with ND. Norrie disease pseudoglioma (NDP) gene was sequenced and clinical examination was performed on patients. A GG dinucleotide insertion in exon 3 (c.240_241insGG) of NDP was detected in all patients. The mutation caused a frameshift and an early stop codon (p.Phe81Glyfs*23). A novel mutation was found in the NDP gene in the affected males of the family. As the mutation was absent in the normal male members of the family, it should be the genetic cause of the disease. © 2017 Optometry Australia.

Molecular analysis of the NDP gene in two families with Norrie disease.

PubMed

Rivera-Vega, M Refugio; Chiñas-Lopez, Silvet; Vaca, Ana Luisa Jimenez; Arenas-Sordo, M Luz; Kofman-Alfaro, Susana; Messina-Baas, Olga; Cuevas-Covarrubias, Sergio Alberto

2005-04-01

To describe the molecular defects in the Norrie disease protein (NDP) gene in two families with Norrie disease (ND). We analysed two families with ND at molecular level through polymerase chain reaction, DNA sequence analysis and GeneScan. Two molecular defects found in the NDP gene were: a missense mutation (265C > G) within codon 97 that resulted in the interchange of arginine by proline, and a partial deletion in the untranslated 3' region of exon 3 of the NDP gene. Clinical findings were more severe in the family that presented the partial deletion. We also diagnosed the carrier status of one daughter through GeneScan; this method proved to be a useful tool for establishing female carriers of ND. Here we report two novel mutations in the NDP gene in Mexican patients and propose that GeneScan is a viable mean of establishing ND carrier status.

Evaluation of the norrie disease gene in a family with incontinentia pigmenti.

PubMed

Shastry, B S; Trese, M T

2000-01-01

Incontinentia pigmenti (IP) is an ectodermal multisystem disorder which can affect dental, ocular, cardiac and neurologic structures. The ocular changes of IP can have a very similar appearance to the retinal detachment of X-linked familial exudative vitreoretinopathy, which has been shown to be caused by the mutations in the Norrie disease gene. Therefore, it is of interest to determine whether similar mutations in the gene can account for the retinal pathology in patients with IP. To test our hypothesis, we have analyzed the entire Norrie disease gene for a family with IP, by single strand conformational polymorphism followed by DNA sequencing. The sequencing data revealed no disease-specific sequence alterations. These data suggest that ocular findings of IP are perhaps associated with different genes and there is no direct relationship between the genotype and phenotype. Copyright 2000 S. Karger AG, Basel

76 FR 72216 - Notice of Lodging of Consent Decree Under the Comprehensive Environmental Response, Compensation...

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2011-11-22

... DEPARTMENT OF JUSTICE Notice of Lodging of Consent Decree Under the Comprehensive Environmental Response, Compensation and Liability Act (``CERCLA'') Consistent with Section 122(d)(2) of CERCLA, 42 U.S.C. 9622(d)(2), notice is hereby given that on November 7, 2011, a proposed Consent Decree in The General...

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Federal Register 2010, 2011, 2012, 2013, 2014

2012-08-22

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Genotype-phenotype variations in five Spanish families with Norrie disease or X-linked FEVR.

PubMed

Riveiro-Alvarez, Rosa; Trujillo-Tiebas, Maria José; Gimenez-Pardo, Ascension; Garcia-Hoyos, Maria; Cantalapiedra, Diego; Lorda-Sanchez, Isabel; Rodriguez de Alba, Marta; Ramos, Carmen; Ayuso, Carmen

2005-09-02

Norrie disease (OMIM 310600) is a rare X-linked disorder characterized by congenital blindness in males. Approximately 40 to 50% of the cases develop deafness and mental retardation. X-linked familial exudative vitreoretinopathy (XL-FEVR) is a hereditary ocular disorder characterized by a failure of peripheral retinal vascularization. Both X-linked disorders are due to mutations in the NDP gene, which encodes a 133 amino acid protein called Norrin, but autosomal recessive (AR) and autosomal dominant (AD) forms of FEVR have also been described. In this study, we report the molecular findings and the related phenotype in five Spanish families affected with Norrie disease or XL-FEVR due to mutations of the NDP gene. The study was conducted in 45 subjects from five Spanish families. These families were clinically diagnosed with Norrie disease or similar conditions. The three exons of the NDP gene were analyzed by automatic DNA sequencing. Haplotype analyses were also performed. Two new nonsense mutations, apart from other mutations previously described in the NDP gene, were found in those patients affected with ND or X-linked FEVR. An important genotype-phenotype variation was found in relation to the different mutations of the NDP gene. In fact, the same mutation may be responsible for different phenotypes. We speculate that there might be other molecular factors that interact in the retina with Norrin, which contribute to the resultant phenotypes.

Ocular phenotypes associated with two mutations (R121W, C126X) in the Norrie disease gene.

PubMed

Kellner, U; Fuchs, S; Bornfeld, N; Foerster, M H; Gal, A

1996-06-01

To describe the ocular phenotypes associated with 2 mutations in the Norrie disease gene including a manifesting carrier. Ophthalmological examinations were performed in 2 affected males and one manifesting carrier. Genomic DNA was analyzed by direct sequencing of the Norrie disease gene. Family I: A 29-year-old male had the right eye enucleated at the age of 3 years. His left eye showed severe temporal dragging of the retina and central scars. Visual acuity was 20/300. DNA analysis revealed a C-to-T transition of the first nucleotide in codon 121 predicting the replacement of arginine-121 by tryptophan (R121W). Both the mother and maternal grandmother carry the same mutation in heterozygous form. Family 2: A 3-month-old boy presented with severe temporal dragging of the retina on both eyes and subsequently developed retinal detachment. Visual acuity was limited to light perception. His mother's left eye was amaurotic and phthitic. Her right eye showed severe retinal dragging, visual acuity was reduced to 20/60. DNA analysis revealed a T-to-A transversion of the third nucleotide in codon 126 creating a stop codon (C126X). The mother and maternal grandmother were carriers. Mutations in the Norrie disease gene can lead to retinal malformations of variable severity both in hemizygous males and manifesting carriers.

Hydrothermal disturbances at the Norris Geyser Basin, Yellowstone National Park (USA) in 2003

NASA Astrophysics Data System (ADS)

Lowenstern, J. B.; Heasler, H.; Smith, R. B.

2003-12-01

The Norris Geyser Basin in north-central Yellowstone National Park (YNP) experienced a series of notable changes during 2003, including formation of new hot springs and fumaroles, renewed activity of dormant geysers and elevated ground temperatures. This abstract provides a short synopsis of the new hydrothermal activity. In 2000, Yellowstone's tallest geyser, Steamboat, erupted after a dormant period of nearly 9 years. It erupted twice in 2002 and then again on 26 March and 27 April 2003. Surges in flux of thermal water preceding the eruptions (preplay) were recorded by a couplet of temperature data loggers placed in the outlet stream. The data indicated pulses of water flow with 1 and ~3 day intervals. On 10 July 2003, a new thermal feature was reported just west of Nymph Lake, ~ 3.5 km northwest of the Norris Museum. A linear series of vigorous fumaroles, about 75 m long had formed in a forested area, ~ 200 m up a hill on the lake's west shore. Fine particles of rock and mineral fragments coated nearby vegetation. Fumarole temperatures were around the local boiling temperature of water (92° C). After two months, somewhat reduced steam emission was accompanied by discharge of ~ 3-10 gallons per minute of near-neutral thermal water. Trees within 4 meters of the lineament were dead and were being slowly combusted. Porkchop Geyser in Norris' Back Basin had been dormant since it exploded in 1989, littering the nearby area with boulders up to over 1 m in diameter. Since that time, its water had remained well below the boiling temperature of water. From 1 April through 1 July `03, the temperature of waters in Porkchop's vent increased continuously from 67° to 88° C. Each Summer, Norris' Back Basin experiences an "annual disturbance" where individual hot springs and geysers typically show anomalous boiling, and have measurable increases in turbidity, acidity and SO4/Cl ratios. The disturbance has been linked to depressurization of the hydrothermal system as the hydrostatic pressure of the snowmelt-fed groundwater table wanes each summer. This year, the "Norris disturbance" is estimated to have begun on 11 July 2003,when pronounced changes were noted, including thermal pools that were boiled to dryness (e.g., Pearl Geyser), creating fumaroles. Porkchop Geyser erupted for the first time since 1989 on 16 July. New mud pots formed along the Back Basin Trail and increased ground temperatures were noted over an 500 x 300 m area. Park staff noted temperatures up to 94° C at 1 cm beneath the ground surface in areas that were previously cool. Vegetation in the area immediately died and began to break down due to the high temperatures. Yellowstone National Park closed the Back Basin Trail to all visitor travel on 22 July 2003. During the first week of August 2003, the Yellowstone Volcano Observatory installed a temporary monitoring network in the Norris Geyser Basin. University of Utah staff, with equipment and personnel made available by IRIS, UNAVCO, USGS and YNP, installed seven broadband seismometers and five continuous GPS receivers. In addition, YNP deployed a series of temperature data-loggers to record changes in flow from thermal features within the Norris Back Basin. The network is intended to identify ground motions associated with fluid flow within the geyser basin that may accompany eruptions of geysers, boiling episodes or events precursory to hydrothermal explosions. The monitoring network will remain operational for between four and twelve weeks.

75 FR 5343 - Notice of Lodging of Consent Decree Under Sections 106, 107 and 113 of the Comprehensive...

Federal Register 2010, 2011, 2012, 2013, 2014

2010-02-02

... DEPARTMENT OF JUSTICE Notice of Lodging of Consent Decree Under Sections 106, 107 and 113 of the Comprehensive Environmental Response, Compensation, and Liability Act of 1980, 42 U.S.C. 9606, 9607 and 9613, as... hazardous substances at or from the Armour Road Superfund Site, located at and adjacent to 2251 Armour Road...

Integration of Enhanced Propagation, Environmental Variability, and Network Performance Models into the InfraMAP Software Toolkit

DTIC Science & Technology

2007-11-01

distribution is unlimited. Z November 2007 Li DTRA01-00-C-0063 David Norris and Robert Gibson Prepared by: BBN Technologies 1300 North 17th Street Suite...TA - CD C.AUT040WS WU - 02091 David E. Norris Robert G. Gibson 7. PERVORMS ONSAIZATIMN RAW(S) AM AURS118" L PIRU OANIZATMO ASPORT...be useful in determining the relative importance with which to treat the predictions. Support of synergy-based localization - Confidence bounds

Norrie-Warburg syndrome: two novel mutations in patients with classical clinical phenotype.

PubMed

Gal, A; Veske, A; Jojart, G; Grammatico, B; Huber, B; Gu, S; del Porto, G; Senyi, K

1996-01-01

Norrie-Warburg syndrome (NWS) is a rare X-linked disorder characterized by blindness, which is invariable, deafness and mental disturbances, which are present occasionally. We describe here two novel mutations, a missense mutation (C126S) and a 1-base pair insertion (insT466/T467), together with a recurrent mutation (M1V), found in patients presenting with the classical clinical phenotype of NWS. All three mutations are likely to result in prominent structural changes of the norrin protein.

Race, gender, and language concordance in the primary care setting.

PubMed

Martin, Brian C; Shi, Leiyu; Ward, Ryan D

2009-01-01

The purpose of this paper is to examine race, gender and language concordance in terms of importance to primary care. The 2003 Medical Expenditure Panel Survey Household Component (MEPS) was used. Four distinguishing primary care attributes and selected measures were operationalized primarily from a sample subset that identified a usual source of care (USC): accessibility to USC; interface between primary care and specialist services; treatment decisions; and preventive services received from the USC. Bivariate and multivariate results are reported. Adjusting for covariates, the following items remained statistically significant: race--choosing primary care physician as USC, USC having office hours, and going to USC for new health problems; gender--choosing primary care physician as USC and USC having office hours; and language--lack of difficulty contacting the USC after hours. However, these items appear to be isolated cases rather than indicators that concordance plays a key role in determining primary care quality. Language barriers/communication issues are the only areas where improvement appears warranted. While the study has strong accessibility and interpersonal relationship measures, service coordination and comprehensiveness indicators are limited. The analyses' cross-sectional nature also poses a problem in drawing causal relationships and conclusive findings. Finally, sample size limitations preclude stratified analyses across racial/ethnic groups, an important consideration as the relationships between concordance and quality may vary across groups. This study indicates that more research is needed in this area to determine future resource allocation and policy direction. The unique contribution of the study is to suggest that race and gender concordance may not accurately predict primary health care quality.

Hedgehog regulates Norrie disease protein to drive neural progenitor self-renewal.

PubMed

McNeill, Brian; Mazerolle, Chantal; Bassett, Erin A; Mears, Alan J; Ringuette, Randy; Lagali, Pamela; Picketts, David J; Paes, Kim; Rice, Dennis; Wallace, Valerie A

2013-03-01

Norrie disease (ND) is a congenital disorder characterized by retinal hypovascularization and cognitive delay. ND has been linked to mutations in 'Norrie Disease Protein' (Ndp), which encodes the secreted protein Norrin. Norrin functions as a secreted angiogenic factor, although its role in neural development has not been assessed. Here, we show that Ndp expression is initiated in retinal progenitors in response to Hedgehog (Hh) signaling, which induces Gli2 binding to the Ndp promoter. Using a combination of genetic epistasis and acute RNAi-knockdown approaches, we show that Ndp is required downstream of Hh activation to induce retinal progenitor proliferation in the retina. Strikingly, Ndp regulates the rate of cell-cycle re-entry and not cell-cycle kinetics, thereby uncoupling the self-renewal and cell-cycle progression functions of Hh. Taken together, we have uncovered a cell autonomous function for Ndp in retinal progenitor proliferation that is independent of its function in the retinal vasculature, which could explain the neural defects associated with ND.

Norrie disease gene: characterization of deletions and possible function.

PubMed

Chen, Z Y; Battinelli, E M; Hendriks, R W; Powell, J F; Middleton-Price, H; Sims, K B; Breakefield, X O; Craig, I W

1993-05-01

Positional cloning experiments have resulted recently in the isolation of a candidate gene for Norrie disease (pseudoglioma; NDP), a severe X-linked neurodevelopmental disorder. Here we report the isolation and analysis of human genomic DNA clones encompassing the NDP gene. The gene spans 28 kb and consists of 3 exons, the first of which is entirely contained within the 5' untranslated region. Detailed analysis of genomic deletions in Norrie patients shows that they are heterogeneous, both in size and in position. By PCR analysis, we found that expression of the NDP gene was not confined to the eye or to the brain. An extensive DNA and protein sequence comparison between the human NDP gene and related genes from the database revealed homology with cysteine-rich protein-binding domains of immediate--early genes implicated in the regulation of cell proliferation. We propose that NDP is a molecule related in function to these genes and may be involved in a pathway that regulates neural cell differentiation and proliferation.

Peripheral retinopathy in offspring of carriers of Norrie disease gene mutations. Possible transplacental effect of abnormal Norrin.

PubMed

Mintz-Hittner, H A; Ferrell, R E; Sims, K B; Fernandez, K M; Gemmell, B S; Satriano, D R; Caster, J; Kretzer, F L

1996-12-01

The Norrie disease (ND) gene (Xp11.3) (McKusick 310600) consists of one untranslated exon and two exons partially translated as the Norrie disease protein (Norrin). Norrin has sequence homology and computer-predicted tertiary structure of a growth factor containing a cystine knot motif, which affects endothelial cell migration and proliferation. Norrie disease (congenital retinal detachment), X-linked primary retinal dysplasia (congenital retinal fold), and X-linked exudative vitreoretinopathy (congenital macular ectopia) are allelic disorders. Blood was drawn for genetic studies from members of two families to test for ND gene mutations. Sixteen unaffected family members were examined ophthalmologically. If any retinal abnormality were identified, fundus photography and fluorescein angiography was performed. Family A had ND (R109stp), and family B had X-linked exudative vitreoretinopathy (R121L). The retinas of 11 offspring of carrier females were examined: three of seven carrier females, three of three otherwise healthy females, and one of one otherwise healthy male had peripheral inner retinal vascular abnormalities. The retinas of five offspring of affected males were examined: none of three carrier females and none of two otherwise healthy males had this peripheral retinal finding. Peripheral inner retinal vascular abnormalities similar to regressed retinopathy of prematurity were identified in seven offspring of carriers of ND gene mutations in two families. These ophthalmologic findings, especially in four genetically healthy offspring, strongly support the hypothesis that abnormal Norrin may have an adverse transplacental (environmental) effect on normal inner retinal vasculogenesis.

Characterization and mapping of the mouse NDP (Norrie disease) locus (Ndp).

PubMed

Battinelli, E M; Boyd, Y; Craig, I W; Breakefield, X O; Chen, Z Y

1996-02-01

Norrie disease is a severe X-linked recessive neurological disorder characterized by congenital blindness with progressive loss of hearing. Over half of Norrie patients also manifest different degrees of mental retardation. The gene for Norrie disease (NDP) has recently been cloned and characterized. With the human NDP cDNA, mouse genomic phage libraries were screened for the homolog of the gene. Comparison between mouse and human genomic DNA blots hybridized with the NDP cDNA, as well as analysis of phage clones, shows that the mouse NDP gene is 29 kb in size (28 kb for the human gene). The organization in the two species is very similar. Both have three exons with similar-sized introns and identical exon-intron boundaries between exon 2 and 3. The mouse open reading frame is 393 bp and, like the human coding sequence, is encoded in exons 2 and 3. The absence of six nucleotides in the second mouse exon results in the encoded protein being two amino acids smaller than its human counterpart. The overall homology between the human and mouse NDP protein is 95% and is particularly high (99%) in exon 3, consistent with the apparent functional importance of this region. Analysis of transcription initiation sites suggests the presence of multiple start sites associated with expression of the mouse NDP gene. Pedigree analysis of an interspecific mouse backcross localizes the mouse NDP gene close to Maoa in the conserved segment, which runs from CYBB to PFC in both human and mouse.

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31 CFR 561.803 - Consultations.

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2013-07-01

... Consultations. In implementing sections 104 and 104A of the Comprehensive Iran Sanctions, Accountability, and Divestment Act of 2010 (Pub. L. 111-195) (22 U.S.C. 8501-8551), as amended by the Iran Threat Reduction and...

31 CFR 561.803 - Consultations.

Code of Federal Regulations, 2014 CFR

2014-07-01

... Consultations. In implementing sections 104 and 104A of the Comprehensive Iran Sanctions, Accountability, and Divestment Act of 2010 (Pub. L. 111-195) (22 U.S.C. 8501-8551), as amended by the Iran Threat Reduction and...

Microdeletion in the X-chromosome and prenatal diagnosis in a family with Norrie disease.

PubMed

Zhu, D P; Antonarakis, S E; Schmeckpeper, B J; Diergaarde, P J; Greb, A E; Maumenee, I H

1989-08-01

We have studied a three-generation family in which Norrie disease is segregating and have performed prenatal diagnosis on the fetus of an obligatory carrier. Deletions at loci DXS7 and DXS77 defined by probes L1.28, L1.28-p59, and pX59 were detected in the affected male. DNA studies of chorionic villus biopsy material indicated that the male fetus had inherited the normal allele from the carrier mother. This prediction was confirmed on eye examination at age 5 months.

Norris Bradbury Tribute by J. Robert Oppenheimer

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

Meade, Roger Allen

Stored on a reel of microfilm are three faint and barely readable letters written on the occasion of Norris Bradbury’s fifteen-year anniversary as Director of the (then) Los Alamos Science Laboratory. The first letter, written by Los Alamos Public Relations Officer John V. Young, asks Oppenheimer to send a “congratulatory message to be read at a ceremony honoring Bradbury’s tenure as Laboratory Director. The second letter is Oppenheimer’s response to Young, and the third is Oppenheimer’s congratulatory message to Bradbury. The three letters are transcribed below.

[Norrie-Wardburg syndrome].

PubMed

Skevas, A; Kastanioudakis, I; Daniilidis, B; Exarchakos, G

1992-10-01

We describe a case of a 25-year old patient with typical Norrie-Warburg Syndrome. From the first year of his life he was found to be blind, with bilateral sensorineural loss of hearing. Audiological examination showed symmetrical moderate bilateral sensorineural hearing loss. His hearing loss was refractory to treatment for the last eight years. Because of timely diagnosis of hearing loss and timely fitting of a hearing aid, the patient could study at school and graduate from university education. Disease carriers who are clinically healthy can be identified only via chromosome analysis.

[Norrie syndrome (author's transl)].

PubMed

Schmitz-Valckenberg, P; Scholz, W

1977-10-01

The Norrie syndrome, an x-chromosomal linked, recessive genetic disease, is described using ophthalmologic and genetic examinations of a family in three generations. The main symptom of this syndrome is retinal detachment with hemorrhages, which generally leads to blindness in early childhood. In addition to this, in 25--35% of the cases mental retardation and hearing problems are found. Special significance is to be attached to the differential diagnosis of this syndrome because the vascular proliferation on the retina is a non-specific, secondary reaction in children, which also occurs symptomatically in several other diseases.

A novel Norrie disease pseudoglioma gene mutation, c.-1_2delAAT, responsible for Norrie disease in a Chinese family.

PubMed

Zhang, Xin-Yu; Jiang, Wei-Ying; Chen, Lu-Ming; Chen, Su-Qin

2013-01-01

To investigate the genetic findings and phenotypic characteristics of a Chinese family with Norrie disease (ND). Molecular genetic analysis and clinical examinations were performed on a Chinese family with ND. Mutations in the Norrie disease pseudoglioma (NDP) gene were detected by direct sequencing. Haplotypes were constructed and compared with the phenotypes in the family. Evolutionary comparisons and mutant open reading frame (ORF) prediction were also undertaken. Two family members with ocular manifestations were diagnosed with ND. No signs of sensorineural hearing loss were observed in either patient, while one of them showed signs of mild mental retardation. A novel heterozygous mutation in the NDP gene, c.-1_2delAAT, was detected in both patients. The mutation and the mutation bearing haplotype co-segregated with the ND phenotype in males and was transmitted from their mothers and/or grandmothers (II:2). The male without ND did not harbor the mutation. The mutation occurred at the highly conserved nucleotides. ORF finder predicted that the mutation would lead to the production of a truncated protein that lacks the first 11 N-terminal amino acids. A novel mutation, c.-1_2delAAT in the NDP gene, was identified in a Chinese family with ND. This mutation caused ND without obvious sensorineural hearing loss. Mental disorder was found in one but not the other patients. The clinical heterogeneity in the family indicated that other genetic variants and epigenetic factors may also play a role in the disease presentation.

Plasma amine oxidase activities in Norrie disease patients with an X-chromosomal deletion affecting monoamine oxidase.

PubMed

Murphy, D L; Sims, K B; Karoum, F; Garrick, N A; de la Chapelle, A; Sankila, E M; Norio, R; Breakefield, X O

1991-01-01

Two individuals with an X-chromosomal deletion were recently found to lack the genes encoding monoamine oxidase type A (MAO-A) and MAO-B. This abnormality was associated with almost total (90%) reductions in the oxidatively deaminated urinary metabolites of the MAO-A substrate, norepinephrine, and with marked (100-fold) increases in an MAO-B substrate, phenylethylamine, confirming systemic functional consequences of the genetic enzyme deficiency. However, urinary concentrations of the deaminated metabolites of dopamine and serotonin (5-HT) were essentially normal. To investigate other deaminating systems besides MAO-A and MAO-B that might produce these metabolites of dopamine and 5-HT, we examined plasma amine oxidase (AO) activity in these two patients and two additional patients with the same X-chromosomal deletion. Normal plasma AO activity was found in all four Norrie disease-deletion patients, in four patients with classic Norrie disease without a chromosomal deletion, and in family members of patients from both groups. Marked plasma amine metabolite abnormalities and essentially absent platelet MAO-B activity were found in all four Norrie disease-deletion patients, but in none of the other subjects in the two comparison groups. These results indicate that plasma AO is encoded by gene(s) independent of those for MAO-A and MAO-B, and raise the possibility that plasma AO, and perhaps the closely related tissue AO, benzylamine oxidase, as well as other atypical AOs or MAOs encoded independently from MAO-A and MAO-B may contribute to the oxidative deamination of dopamine and 5-HT in humans.

A novel Norrie disease pseudoglioma gene mutation, c.-1_2delAAT, responsible for Norrie disease in a Chinese family

PubMed Central

Zhang, Xin-Yu; Jiang, Wei-Ying; Chen, Lu-Ming; Chen, Su-Qin

2013-01-01

AIM To investigate the genetic findings and phenotypic characteristics of a Chinese family with Norrie disease (ND). METHODS Molecular genetic analysis and clinical examinations were performed on a Chinese family with ND. Mutations in the Norrie disease pseudoglioma (NDP) gene were detected by direct sequencing. Haplotypes were constructed and compared with the phenotypes in the family. Evolutionary comparisons and mutant open reading frame (ORF) prediction were also undertaken. RESULTS Two family members with ocular manifestations were diagnosed with ND. No signs of sensorineural hearing loss were observed in either patient, while one of them showed signs of mild mental retardation. A novel heterozygous mutation in the NDP gene, c.-1_2delAAT, was detected in both patients. The mutation and the mutation bearing haplotype co-segregated with the ND phenotype in males and was transmitted from their mothers and/or grandmothers (II:2). The male without ND did not harbor the mutation. The mutation occurred at the highly conserved nucleotides. ORF finder predicted that the mutation would lead to the production of a truncated protein that lacks the first 11 N-terminal amino acids. CONCLUSION A novel mutation, c.-1_2delAAT in the NDP gene, was identified in a Chinese family with ND. This mutation caused ND without obvious sensorineural hearing loss. Mental disorder was found in one but not the other patients. The clinical heterogeneity in the family indicated that other genetic variants and epigenetic factors may also play a role in the disease presentation. PMID:24392318

The Norrie disease gene maps to a 150 kb region on chromosome Xp11.3.

PubMed

Sims, K B; Lebo, R V; Benson, G; Shalish, C; Schuback, D; Chen, Z Y; Bruns, G; Craig, I W; Golbus, M S; Breakefield, X O

1992-05-01

Norrie disease is a human X-linked recessive disorder of unknown etiology characterized by congenital blindness, sensory neural deafness and mental retardation. This disease gene was previously linked to the DXS7 (L1.28) locus and the MAO genes in band Xp11.3. We report here fine physical mapping of the obligate region containing the Norrie disease gene (NDP) defined by a recombination and by the smallest submicroscopic chromosomal deletion associated with Norrie disease identified to date. Analysis, using in addition two overlapping YAC clones from this region, allowed orientation of the MAOA and MAOB genes in a 5'-3'-3'-5' configuration. A recombination event between a (GT)n polymorphism in intron 2 of the MAOB gene and the NDP locus, in a family previously reported to have a recombination between DXS7 and NDP, delineates a flanking marker telomeric to this disease gene. An anonymous DNA probe, dc12, present in one of the YACs and in a patient with a submicroscopic deletion which includes MAOA and MAOB but not L1.28, serves as a flanking marker centromeric to the disease gene. An Alu-PCR fragment from the right arm of the MAO YAC (YMAO.AluR) is not deleted in this patient and also delineates the centromeric extent of the obligate disease region. The apparent order of these loci is telomere ... DXS7-MAOA-MAOB-NDP-dc12-YMAO.AluR ... centromere. Together these data define the obligate region containing the NDP gene to a chromosomal segment less than 150 kb.

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..., including Exide Illinois, Inc., Exide Delaware, LLC, RBD Liquidation, LLC, Dixie Metals Company, and Refined... Conservation and Recovery Act (``RCRA''), 42 U.S.C. 6973, for the following 21 sites: (1) Hamburg Lead...

X-linked recessive primary retinal dysplasia is linked to the Norrie disease locus.

PubMed

Ravia, Y; Braier-Goldstein, O; Bat-Miriam, K M; Erlich, S; Barkai, G; Goldman, B

1993-08-01

X-linked primary retinal dysplasia (PRD) refers to an abnormal proliferation of retinal tissue causing either its neural elements or its glial tissue to form folds, giving rise to gliosis. A Jewish family of oriental origin was previously reported by Godel and Goodman, in which a total of five males suffer from different degrees of blindness. The authors postulated that the described findings are distinguished from Norrie disease, since in this case no clinical findings, other than those associated with the eyes, were noticed in the affected males. In addition, two of the carrier females exhibit minimal eye changes. We have performed linkage analysis of the family using the L1.28, p58-1 and m27 beta probes, and DXS426 and MAOB associated microsatellites. Our results map the gene responsible for the disorder between the MAOB and DXS426, m27 beta and p58-1 loci, on the short arm of the X chromosome at Xp11.3, which suggest the possibility that the same gene is responsible for both primary retinal dysplasia and Norrie disease.

NDP gene mutations in 14 French families with Norrie disease.

PubMed

Royer, Ghislaine; Hanein, Sylvain; Raclin, Valérie; Gigarel, Nadine; Rozet, Jean-Michel; Munnich, Arnold; Steffann, Julie; Dufier, Jean-Louis; Kaplan, Josseline; Bonnefont, Jean-Paul

2003-12-01

Norrie disease is a rare X-inked recessive condition characterized by congenital blindness and occasionally deafness and mental retardation in males. This disease has been ascribed to mutations in the NDP gene on chromosome Xp11.1. Previous investigations of the NDP gene have identified largely sixty disease-causing sequence variants. Here, we report on ten different NDP gene allelic variants in fourteen of a series of 21 families fulfilling inclusion criteria. Two alterations were intragenic deletions and eight were nucleotide substitutions or splicing variants, six of them being hitherto unreported, namely c.112C>T (p.Arg38Cys), c.129C>G (p.His43Gln), c.133G>A (p.Val45Met), c.268C>T (p.Arg90Cys), c.382T>C (p.Cys128Arg), c.23479-1G>C (unknown). No NDP gene sequence variant was found in seven of the 21 families. This observation raises the issue of misdiagnosis, phenocopies, or existence of other X-linked or autosomal genes, the mutations of which would mimic the Norrie disease phenotype. Copyright 2003 Wiley-Liss, Inc.

Using noble gases measured in spring discharge to trace hydrothermal processes in the Norris Geyser Basin, Yellowstone National Park, U.S.A.

USGS Publications Warehouse

Gardner, W.P.; Susong, D.D.; Solomon, D.K.; Heasler, H.P.

2010-01-01

Dissolved noble gas concentrations in springs are used to investigate boiling of hydrothermal water and mixing of hydrothermal and shallow cool water in the Norris Geyser Basin area. Noble gas concentrations in water are modeled for single stage and continuous steam removal. Limitations on boiling using noble gas concentrations are then used to estimate the isotopic effect of boiling on hydrothermal water, allowing the isotopic composition of the parent hydrothermal water to be determined from that measured in spring. In neutral chloride springs of the Norris Geyser Basin, steam loss since the last addition of noble gas charged water is less than 30% of the total hydrothermal discharge, which results in an isotopic shift due to boiling of ?? 2.5% ??D. Noble gas concentrations in water rapidly and predictably change in dual phase systems, making them invaluable tracers of gas-liquid interaction in hydrothermal systems. By combining traditional tracers of hydrothermal flow such as deuterium with dissolved noble gas measurements, more complex hydrothermal processes can be interpreted. ?? 2010 Elsevier B.V.

Cerebroretinal microangiopathy with calcifications and cysts associated with CTC1 and NDP mutations.

PubMed

Romaniello, Romina; Arrigoni, Filippo; Citterio, Andrea; Tonelli, Alessandra; Sforzini, Cinzia; Rizzari, Carmelo; Pessina, Marco; Triulzi, Fabio; Bassi, Maria Teresa; Borgatti, Renato

2013-12-01

Mutations in the conserved telomere maintenance component 1 (CTC1) gene were recently described in Coats plus syndrome and in cerebroretinal microangiopathy with calcifications and cysts. Norrie disease protein (NDP) gene was found mutated in Norrie disease, in Familial Exudative Vitreoretinopathy, and in Coats syndrome. Here we describe a boy affected by Norrie disease who developed typical features of cerebroretinal microangiopathy with calcifications and cysts. Direct sequencing of the CTC1 and NDP genes in this patient shows the presence of compound heterozygosity for 2 mutations in CTC1 (c.775G>A, pV259M and a novel microdeletion c.1213delG) and a missense mutation in the NDP gene (c.182T>C, p.L61P). Based on these genetic findings and on the expression of both genes in endothelial cells, we postulate that microangiopathy might be a primary underlying pathologic abnormality in cerebroretinal microangiopathy with calcifications and cysts. This hypothesis is further supported by magnetic resonance imaging (MRI) data showing multiple minute calcifications in the deep gray nuclei and in terminal arteriolar zones.

Two novel mutations in the Norrie disease gene associated with the classical ocular phenotype.

PubMed

Caballero, M; Veske, A; Rodriguez, J J; Lugo, N; Schroeder, B; Hesse, L; Gal, A

1996-12-01

Norrie disease (ND) is a rare X-linked recessive disorder characterized by congenital blindness due to a degenerative and proliferative dysplasia of the neuroretina and, occasionally, by deafness and mental handicap. Here, we report two novel mutations detected in patients with the classical eye features of ND. Both the one-base pair insertion in exon II (544/545 insA) and the two-base pair deletion in the start codon (418delTG) of the ND gene predict a functional 'null allele', i.e. the complete absence of the corresponding gene product.

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Sustainable transportation according to certification systems: A viability analysis based on neighborhood size and context relevance

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

Gouda, Amr Ah., E-mail: amr.gouda@eng.asu.edu.eg; Architecture Department, Faculty of Engineering, Ain Shams University, 1 Sarayat St., Abassia, Cairo; Masoumi, Houshmand E.

Urban sustainability certification (USC) systems comprehensively assess and benchmark the sustainability of neighborhoods, communities, etc. However, it is important to understand what USCs mean by neighborhoods and communities in terms of definition and their certified developments' size. This study focuses on sustainable transportation and its measures in USCs in order to discern: how relevant are these measures to the varied local conditions, especially in developing countries? And whether they can generate palpable benefits vis-à-vis the certified developments' site areas. Taking descriptive analysis methods, this study focuses on four prominent USCs, namely, LEED for Neighborhood Development, BREEAM Communities, CASBEE for Urbanmore » Development, and The Pearl Community Rating System. The four USCs prescribed multiple measures for sustainable transportation, particularly in favor of active transportation and public transportation. However, the relatively small size of their citified developments and the varying transportation conditions among different contexts attenuate the viability and relevance of the advocated measures for both modes. In order to yield more benefits, USCs should underscore the integrative nature of neighborhoods and communities, and transportation within their prescribed criteria and measures. Moreover, sustainable transportation as a theme should be tailored to the local conditions rather than being adopted or adapted from global USCs. - Highlights: • Transportation measures in four prominent certification systems were investigated. • Most certified neighborhoods, communities and developments have small site areas. • Certifications are insensitive to realities of transport in developing countries. • Benefits from advocating active transport only within certified areas are minimal. • Certifications devalue the integrative nature of neighborhoods and transportation.« less

Novel mutations in Norrie disease gene in Japanese patients with Norrie disease and familial exudative vitreoretinopathy.

PubMed

Kondo, Hiroyuki; Qin, Minghui; Kusaka, Shunji; Tahira, Tomoko; Hasebe, Haruyuki; Hayashi, Hideyuki; Uchio, Eiichi; Hayashi, Kenshi

2007-03-01

To search for mutations in the Norrie disease gene (NDP) in Japanese patients with familial exudative vitreoretinopathy (FEVR) and Norrie disease (ND) and to delineate the mutation-associated clinical features. Direct sequencing after polymerase chain reaction of all exons of the NDP gene was performed on blood collected from 62 probands (31 familial and 31 simplex) with FEVR, from 3 probands with ND, and from some of their family members. The clinical symptoms and signs in the patients with mutations were assessed. X-inactivation in the female carriers was examined in three FEVR families by using leukocyte DNA. Four novel mutations-I18K, K54N, R115L, and IVS2-1G-->A-and one reported mutation, R97P, in the NDP gene were identified in six families. The severity of vitreoretinopathy varied among these patients. Three probands with either K54N or R115L had typical features of FEVR, whereas the proband with R97P had those of ND. Families with IVS2-1G-->A exhibited either ND or FEVR characteristics. A proband with I18K presented with significant phenotypic heterogeneity between the two eyes. In addition, affected female carriers in a family harboring the K54N mutation presented with different degrees of vascular abnormalities in the periphery of the retina. X-inactivation profiles indicated that the skewing was not significantly different between affected and unaffected women. These observations indicate that mutations of the NDP gene can cause ND and 6% of FEVR cases in the Japanese population. The X-inactivation assay with leukocytes may not be predictive of the presence of a mutation in affected female carriers.

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Expanding roles in a library-based bioinformatics service program: a case study

PubMed Central

Li, Meng; Chen, Yi-Bu; Clintworth, William A

2013-01-01

Question: How can a library-based bioinformatics support program be implemented and expanded to continuously support the growing and changing needs of the research community? Setting: A program at a health sciences library serving a large academic medical center with a strong research focus is described. Methods: The bioinformatics service program was established at the Norris Medical Library in 2005. As part of program development, the library assessed users' bioinformatics needs, acquired additional funds, established and expanded service offerings, and explored additional roles in promoting on-campus collaboration. Results: Personnel and software have increased along with the number of registered software users and use of the provided services. Conclusion: With strategic efforts and persistent advocacy within the broader university environment, library-based bioinformatics service programs can become a key part of an institution's comprehensive solution to researchers' ever-increasing bioinformatics needs. PMID:24163602

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... PROTECTION PROVISIONS OF SIX ENVIRONMENTAL STATUTES AND SECTION 211 OF THE ENERGY REORGANIZATION ACT OF 1974... of Labor has been given responsibility pursuant to the following Federal statutes: Safe Drinking...; Energy Reorganization Act of 1974, 42 U.S.C. 5851; and Comprehensive Environmental Response, Compensation...

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... PROTECTION PROVISIONS OF SIX ENVIRONMENTAL STATUTES AND SECTION 211 OF THE ENERGY REORGANIZATION ACT OF 1974... of Labor has been given responsibility pursuant to the following Federal statutes: Safe Drinking...; Energy Reorganization Act of 1974, 42 U.S.C. 5851; and Comprehensive Environmental Response, Compensation...

7 CFR 3430.702 - Definitions.

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2011-01-01

... biomass feedstock. DOE means the Department of Energy. Institutions of higher education has the meaning given the term in section 102 of the Higher Education Act of 1965 (20 U.S.C. 1002(a)). Intermediate... assessment means the comprehensive examination of a product's environmental and economic aspects and...

KENNEDY SPACE CENTER, FLA. - James Harrison (left), Jack Nowling (center) and Amy Norris (right) pack up part of the debris stored in the Columbia Debris Hangar. An area of the Vehicle Assembly Building is being prepared to store the debris. About 83,000 pieces were shipped to KSC during search and recovery efforts in East Texas.

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2003-09-10

KENNEDY SPACE CENTER, FLA. - James Harrison (left), Jack Nowling (center) and Amy Norris (right) pack up part of the debris stored in the Columbia Debris Hangar. An area of the Vehicle Assembly Building is being prepared to store the debris. About 83,000 pieces were shipped to KSC during search and recovery efforts in East Texas.

KENNEDY SPACE CENTER, FLA. - James Harrison (left), Jack Nowling (center) and Amy Norris (right) pack up some of the debris stored in the Columbia Debris Hangar. About 83,000 pieces were shipped to KSC during search and recovery efforts in East Texas. An area of the Vehicle Assembly Building is being prepared to store the debris.

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2003-09-10

KENNEDY SPACE CENTER, FLA. - James Harrison (left), Jack Nowling (center) and Amy Norris (right) pack up some of the debris stored in the Columbia Debris Hangar. About 83,000 pieces were shipped to KSC during search and recovery efforts in East Texas. An area of the Vehicle Assembly Building is being prepared to store the debris.

The Wnt signaling pathway in familial exudative vitreoretinopathy and Norrie disease.

PubMed

Warden, Scott M; Andreoli, Christopher M; Mukai, Shizuo

2007-01-01

The Wnt signaling pathway is highly conserved among species and has an important role in many cell biological processes throughout the body. This signaling cascade is involved in regulating ocular growth and development, and recent findings indicate that this is particularly true in the retina. Mutations involving different aspects of the Wnt signaling pathway are being linked to several diseases of retinal development. The aim of this article is to first review the Wnt signaling pathway. We will then describe two conditions, familial exudative vitreoretinopathy (FEVR) and Norrie disease (ND), which have been shown to be caused in part by defects in the Wnt signaling cascade.

Clinical reinvestigation and linkage analysis in the family with Episkopi blindness (Norrie disease).

PubMed

Wolff, G; Mayerová, A; Wienker, T F; Atalianis, P; Ioannou, P; Warburg, M

1992-11-01

We present the results of a clinical and genetic reinvestigation of the Cypriot family affected by an X chromosomally inherited eye disease originally published by Taylor et al, who coined the term Episkopi blindness. The pedigree was extended to 160 members, including 16 affected males out of 48 males at risk for the disease, most of whom were seen by one of us (PA). Affected males are blind with no associated symptoms and apparently are not mentally retarded. Thirty-nine family members agreed to blood sampling for genetic investigations. RFLP analysis was performed using probes from the region known to be deleted in some Norrie patients and polymorphic markers (DXS77, DXS7, MAOA, DXS255) from the proximal short arm of the X chromosome. There was no deletion for any of the probes in the affected males. Linkage analysis yielded positive lod scores for all informative markers (Z (DXS255, theta = 0) = 6.54, Z (MAOA, theta = 0) = 2.23, Z (DXS7, theta = 0) = 2.13). Thus, the conclusion that Episkopi blindness and Norrie disease (NDP, MIM *310600) are the same entity based on clinical evidence is now reinforced by gene mapping.

Identification of A Novel Missense Mutation in The Norrie Disease Gene: The First Molecular Genetic Analysis and Prenatal Diagnosis of Norrie Disease in An Iranian Family.

PubMed

Talebi, Farah; Ghanbari Mardasi, Farideh; Mohammadi Asl, Javad; Lashgari, Ali; Farhadi, Freidoon

2018-07-01

Norrie disease (ND) is a rare X-linked recessive disorder, which is characterized by congenital blindness and, in several cases, accompanied with mental retardation and deafness. ND is caused by mutations in NDP, located on the proximal short arm of the X chromosome (Xp11.3). The disease has been observed in many ethnic groups worldwide, however, no such case has been reported from Iran. In this study, we present the molecular analysis of two patients with ND and the subsequent prenatal diagnosis. Screening of NDP identified a hemizygous missense mutation (p.Ser133Cys) in the affected male siblings of the family. The mother was the carrier for the mutation (p.Ser133Cys). In a subsequent chorionic amniotic pregnancy, we carried out prenatal diagnosis by sequencing NDP in the chorionic villi sample at 11 weeks of gestation. The fetus was carrying the mutation and thus unaffected. This is the first mutation report and prenatal diagnosis of an Iranian family with ND, and highlights the importance of prenatal diagnostic screening of this congenital disorder and relevant genetic counseling. Copyright© by Royan Institute. All rights reserved.

Norrie disease: extraocular clinical manifestations in 56 patients.

PubMed

Smith, Sharon E; Mullen, Thomas E; Graham, Dionne; Sims, Katherine B; Rehm, Heidi L

2012-08-01

Norrie disease (ND) is an X-linked recessive disorder characterized by congenital blindness, progressive sensorineural hearing loss and cognitive impairment. The ocular phenotype has been well described, while the extraocular manifestations of the disorder are not well understood. We present the data from the Norrie Disease Registry, which consists of 56 patients with detailed clinical histories and genotype data. This study represents the largest, detailed investigation into the phenotypic spectrum of ND to date and more importantly expands knowledge of the extraocular clinical manifestations. We identify several novel aspects of the syndrome that will improve the management of these patients. In particular, we expand our understanding of the neurologic manifestations in ND and identify a chronic seizure disorder in approximately 10% of all patients. In addition, details of the hearing phenotype are described including the median age of onset (12 years of age) and how genotype affects onset. Moreover, we find vascular disease to be a significant component of ND; and vascular health should be, in the future, a component of patient clinical care. In summary, the results expand our understanding of the phenotypic variability and genotypic heterogeneity in ND patients. Copyright © 2012 Wiley Periodicals, Inc.

Clinical reinvestigation and linkage analysis in the family with Episkopi blindness (Norrie disease).

PubMed Central

Wolff, G; Mayerová, A; Wienker, T F; Atalianis, P; Ioannou, P; Warburg, M

1992-01-01

We present the results of a clinical and genetic reinvestigation of the Cypriot family affected by an X chromosomally inherited eye disease originally published by Taylor et al, who coined the term Episkopi blindness. The pedigree was extended to 160 members, including 16 affected males out of 48 males at risk for the disease, most of whom were seen by one of us (PA). Affected males are blind with no associated symptoms and apparently are not mentally retarded. Thirty-nine family members agreed to blood sampling for genetic investigations. RFLP analysis was performed using probes from the region known to be deleted in some Norrie patients and polymorphic markers (DXS77, DXS7, MAOA, DXS255) from the proximal short arm of the X chromosome. There was no deletion for any of the probes in the affected males. Linkage analysis yielded positive lod scores for all informative markers (Z (DXS255, theta = 0) = 6.54, Z (MAOA, theta = 0) = 2.23, Z (DXS7, theta = 0) = 2.13). Thus, the conclusion that Episkopi blindness and Norrie disease (NDP, MIM *310600) are the same entity based on clinical evidence is now reinforced by gene mapping. Images PMID:1453434

75 FR 67433 - Federal Fiscal Year 2011 Annual List of Certifications and Assurances for Federal Transit...

Federal Register 2010, 2011, 2012, 2013, 2014

2010-11-02

...Pursuant to 49 U.S.C. 5323(n), FTA is authorized to consolidate the certifications and assurances required by Federal law or regulations for its programs into a single document. FTA is also required by 49 U.S.C. 5323(n) to publish a list of those certifications and assurances annually. Appendix A of this Notice contains the comprehensive compilation of FTA's Certifications and Assurances applicable to the various Federal assistance programs that FTA will administer during Federal FY 2011. FTA's Certifications and Assurances for Federal FY 2011 reflect Federal statutory, regulatory, and programmatic changes that have now become effective.

23 CFR 650.305 - Definitions.

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2010-04-01

... in a comprehensive training course). Critical finding. A structural or safety related deficiency that... fulfilled education and experience requirements and passed rigorous exams that, under State licensure laws.... Public road. The term “public road” is defined in 23 U.S.C. 101(a)(27). Quality assurance (QA). The use...

34 CFR 656.4 - What types of Centers receive grants?

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Vascular defects and sensorineural deafness in a mouse model of Norrie disease.

PubMed

Rehm, Heidi L; Zhang, Duan-Sun; Brown, M Christian; Burgess, Barbara; Halpin, Chris; Berger, Wolfgang; Morton, Cynthia C; Corey, David P; Chen, Zheng-Yi

2002-06-01

Norrie disease is an X-linked recessive syndrome of blindness, deafness, and mental retardation. A knock-out mouse model with an Ndp gene disruption was studied. We examined the hearing phenotype, including audiological, histological, and vascular evaluations. As is seen in humans, the mice had progressive hearing loss leading to profound deafness. The primary lesion was localized to the stria vascularis, which houses the main vasculature of the cochlea. Fluorescent dyes showed an abnormal vasculature in this region and eventual loss of two-thirds of the vessels. We propose that one of the principal functions of norrin in the ear is to regulate the interaction of the cochlea with its vasculature.

Effect of Having a Usual Source of Care on Health Care Outcomes Among Children With Serious Emotional Disturbance.

PubMed

Witt, Whitney P; Fullerton, Catherine A; Chow, Clifton; Gokhale, Manjusha; Naeger, Sarah; Walsh, Christine; Karnell, Lucy

To determine the influence of a usual source of care (USC) on health care utilization, expenditures, and quality for Medicaid-insured children and adolescents with a serious emotional disturbance (SED). Administrative claims data for 2011-2012 were extracted from the Truven Health MarketScan Multi-State Medicaid Research Database for 286,585 children and adolescents with a primary diagnosis of SED. We used propensity score-adjusted multivariate regressions to determine whether having a USC had a significant effect on utilization and expenditures for high-cost services that are considered potentially avoidable with appropriate outpatient care: physical and behavioral health inpatient admissions, emergency department (ED) visits, and hospital readmissions. Propensity score-adjusted regressions indicated that children with a USC had fewer inpatient admissions related to behavioral health (adjusted odds ratio [AOR] = 0.87; 95% confidence interval [CI], 0.79-0.97) and physical health (AOR = 0.91; 95% CI, 0.89-0.93) and lower expenditures for behavioral health inpatient admissions, physical health ED visits, and readmissions. Having a USC also was associated with a higher likelihood of receiving quality health care for 4 physical health and 2 behavioral health measures. Having a USC improved the health care of Medicaid-insured children and adolescents with an SED. However, despite having insurance, approximately one-fourth of this patient population did not appear to have a USC. This information can be used in developing programs that encourage connections with comprehensive health care that provides coordination among various providers. Copyright © 2016 Academic Pediatric Association. Published by Elsevier Inc. All rights reserved.

Evolution of geothermal fluids deduced from chemistry plots: Yellowstone National Park (U.S.A.)

USGS Publications Warehouse

Mazor, E.; Thompson, J.M.

1982-01-01

Large amounts of chemical data, obtained in geothermal fields, may readily be sorted-out by the aid of a simple set of graphs that provide a clear over-all picture and facilitate the understanding of geochemical processes taking place. As a case study, data from several hundred samples of the thermal springs at the well-known Yellowstone National Park are discussed. The pattern obtained seems to indicate: (1) geochemical similarity between the spring groups of Heart Lake, Shoshone, Upper, Midway, Lower and Norris Geyser Basins, i.e., a geochemical uniformity of major spring groups located over 40 km apart; (2) these groups may be described as originating from a common fluid, most resembling the composition of Norris waters, accompanied by CO2, and other volatiles, that react with igneous rocks, forming local variations; (3) the secondary reactions occur at (medium) depth, before the ascent to the surface; (4) extensive concentration-dilution processes occur during the ascent to the surface. The water of the Mammoth group may be described as originating from the same Norris-like fluid that has been diluted (low Na and Cl contents) and intensively reacted with carbonaceous rocks, thus gaining in Ca, Mg, SO4, and HCO3. ?? 1982.

A novel nonsense mutation in the NDP gene in a Chinese family with Norrie disease.

PubMed

Liu, Deyuan; Hu, Zhengmao; Peng, Yu; Yu, Changhong; Liu, Yalan; Mo, Xiaoyun; Li, Xiaoping; Lu, Lina; Xu, Xiaojuan; Su, Wei; Pan, Qian; Xia, Kun

2010-12-08

Norrie disease (ND), a rare X-linked recessive disorder, is characterized by congenital blindness and, occasionally, mental retardation and hearing loss. ND is caused by the Norrie Disease Protein gene (NDP), which codes for norrin, a cysteine-rich protein involved in ocular vascular development. Here, we report a novel mutation of NDP that was identified in a Chinese family in which three members displayed typical ND symptoms and other complex phenotypes, such as cerebellar atrophy, motor disorders, and mental disorders. We conducted an extensive clinical examination of the proband and performed a computed tomography (CT) scan of his brain. Additionally, we performed ophthalmic examinations, haplotype analyses, and NDP DNA sequencing for 26 individuals from the proband's extended family. The proband's computed tomography scan, in which the fifth ventricle could be observed, indicated cerebellar atrophy. Genome scans and haplotype analyses traced the disease to chromosome Xp21.1-p11.22. Mutation screening of the NDP gene identified a novel nonsense mutation, c.343C>T, in this region. Although recent research has shown that multiple different mutations can be responsible for the ND phenotype, additional research is needed to understand the mechanism responsible for the diverse phenotypes caused by mutations in the NDP gene.

Formaldehyde as a carbon and electron shuttle between autotroph and heterotroph populations in acidic hydrothermal vents of Norris Geyser Basin, Yellowstone National Park.

PubMed

Moran, James J; Whitmore, Laura M; Isern, Nancy G; Romine, Margaret F; Riha, Krystin M; Inskeep, William P; Kreuzer, Helen W

2016-05-01

The Norris Geyser Basin in Yellowstone National Park contains a large number of hydrothermal systems, which host microbial populations supported by primary productivity associated with a suite of chemolithotrophic metabolisms. We demonstrate that Metallosphaera yellowstonensis MK1, a facultative autotrophic archaeon isolated from a hyperthermal acidic hydrous ferric oxide (HFO) spring in Norris Geyser Basin, excretes formaldehyde during autotrophic growth. To determine the fate of formaldehyde in this low organic carbon environment, we incubated native microbial mat (containing M. yellowstonensis) from a HFO spring with (13)C-formaldehyde. Isotopic analysis of incubation-derived CO2 and biomass showed that formaldehyde was both oxidized and assimilated by members of the community. Autotrophy, formaldehyde oxidation, and formaldehyde assimilation displayed different sensitivities to chemical inhibitors, suggesting that distinct sub-populations in the mat selectively perform these functions. Our results demonstrate that electrons originally resulting from iron oxidation can energetically fuel autotrophic carbon fixation and associated formaldehyde excretion, and that formaldehyde is both oxidized and assimilated by different organisms within the native microbial community. Thus, formaldehyde can effectively act as a carbon and electron shuttle connecting the autotrophic, iron oxidizing members with associated heterotrophic members in the HFO community.

Persistent hyperplastic primary vitreous: congenital malformation of the eye.

PubMed

Shastry, Barkur S

2009-12-01

Persistent hyperplastic primary vitreous (PHPV), also known as persistent fetal vasculature, is a rare congenital developmental malformation of the eye, caused by the failure of regression of the primary vitreous. It is divided into anterior and posterior types and is characterized by the presence of a vascular membrane located behind the lens. The condition can be of an isolated type or can occur with other ocular disorders. Most cases of PHPV are sporadic, but it can be inherited as an autosomal dominant or recessive trait. Inherited PHPV also occurs in several breeds of dogs and cats. In a limited number of cases, Norrie disease and FZD4 genes are found to be mutated in unilateral and bilateral PHPV. These genes when mutated also cause Norrie disease pseudoglioma and familial exudative vitreoretinopathy that share some of the clinical features with PHPV. Mice lacking arf and p53 tumour suppressor genes as well as Norrie disease pseudoglioma and LRP5 genes suggest that these genes are needed for hyaloid vascular regression. These experiments also indicate that abnormalities in normal apoptosis and defects in Wnt signalling pathway may be responsible for the pathogenesis of PHPV. Identification of other candidate genes in the future may provide a better understanding of the pathogenesis of the condition that may lead to a better therapeutic approach and better management.

Formaldehyde as a carbon and electron shuttle between autotroph and heterotroph populations in acidic hydrothermal vents of Norris Geyser Basin, Yellowstone National Park

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

Moran, James J.; Whitmore, Laura M.; Isern, Nancy G.

The Norris Geyser Basin in Yellowstone National Park contains a large number of hydrothermal systems, which host microbial populations supported by primary productivity associated with a suite of chemolithotrophic metabolisms. We demonstrate that Metallosphaera yellowstonesis MK1, a facultative autotrophic archaeon isolated from a hyperthermal acidic hydrous ferric oxide (HFO) spring in Norris Geyser Basin, excretes formaldehyde during autotrophic growth. To determine the fate of formaldehyde in this low organic carbon environment, we incubated native microbial mat (containing M. yellowstonensis) from a HFO spring with 13C-formaldehyde. Isotopic analysis of incubation-derived CO2 and biomass showed that formaldehyde was both oxidized and assimilatedmore » by members of the community. Autotrophy, formaldehyde oxidation, and formaldehyde assimilation displayed different sensitivities to chemical inhibitors, suggesting that distinct sub-populations in the mat selectively perform these functions. Our results demonstrate that electrons originally resulting from iron oxidation can energetically fuel autotrophic carbon fixation and associated formaldehyde excretion, and that formaldehyde is both oxidized and assimilated by different organisms within the native microbial community. Thus, formaldehyde can effectively act as a carbon and electron shuttle connecting the autotrophic, iron oxidizing members with associated heterotrophic members in the HFO community.« less

A novel nonsense mutation in the NDP gene in a Chinese family with Norrie disease

PubMed Central

Liu, Deyuan; Hu, Zhengmao; Peng, Yu; Yu, Changhong; Liu, Yalan; Mo, Xiaoyun; Li, Xiaoping; Lu, Lina; Xu, Xiaojuan; Su, Wei; Pan, Qian

2010-01-01

Purpose Norrie disease (ND), a rare X-linked recessive disorder, is characterized by congenital blindness and, occasionally, mental retardation and hearing loss. ND is caused by the Norrie Disease Protein gene (NDP), which codes for norrin, a cysteine-rich protein involved in ocular vascular development. Here, we report a novel mutation of NDP that was identified in a Chinese family in which three members displayed typical ND symptoms and other complex phenotypes, such as cerebellar atrophy, motor disorders, and mental disorders. Methods We conducted an extensive clinical examination of the proband and performed a computed tomography (CT) scan of his brain. Additionally, we performed ophthalmic examinations, haplotype analyses, and NDP DNA sequencing for 26 individuals from the proband’s extended family. Results The proband’s computed tomography scan, in which the fifth ventricle could be observed, indicated cerebellar atrophy. Genome scans and haplotype analyses traced the disease to chromosome Xp21.1-p11.22. Mutation screening of the NDP gene identified a novel nonsense mutation, c.343C>T, in this region. Conclusions Although recent research has shown that multiple different mutations can be responsible for the ND phenotype, additional research is needed to understand the mechanism responsible for the diverse phenotypes caused by mutations in the NDP gene. PMID:21179243

Tectonic significance of porosity and permeability regimes in the red beds formations of the South Georgia Rift Basin

NASA Astrophysics Data System (ADS)

Akintunde, Olusoga M.; Knapp, Camelia C.; Knapp, James H.

2014-09-01

A simple, new porosity/permeability-depth profile was developed from available laboratory measurements on Triassic sedimentary red beds (sandstone) from parts of the South Georgia Rift (SGR) basin in order to investigate the feasibility for long-term CO2 storage. The study locations were: Sumter, Berkeley, Dunbarton, Clubhouse Crossroad-3 (CC-3) and Norris Lightsey wells. As expected, both porosity and permeability show changes with depth at the regional scale that was much greater than at local scale. The significant changes in porosity and permeability with depth suggest a highly compacted, deformed basin, and potentially, a history of uplift and erosion. The permeability is generally low both at shallow (less than 1826 ft/556.56 m) and deeper depths (greater than 1826 ft/556.56 m). Both porosity and permeability follow the normal trend, decreasing linearly with depth for most parts of the study locations with the exception of the Norris Lightsey well. A petrophysical study on a suite of well logs penetrating the Norris Lightsey red beds at depths sampled by the core-derived laboratory measurements shows an abnormal shift (by 50%) in the acoustic travel time and/or in the sonic-derived P-wave velocity that indicates possible faulting or fracturing at depth. The departure of the Norris Lightsey's porosities and permeabilities from the normal compaction trend may be a consequence of the existence of a fault/fracture controlled abnormal pressure condition at depth. The linear and non-linear behaviors of the porosity/permeability distribution throughout the basin imply the composition of the SGR red beds, and by extension analog/similar Triassic-Jurassic formations within the Eastern North American Margin have been altered by compaction, uplift, erosion and possible faulting that have shaped the evolution of these Triassic formations following the major phase of rifting.

Probability and surprisal in auditory comprehension of morphologically complex words.

PubMed

Balling, Laura Winther; Baayen, R Harald

2012-10-01

Two auditory lexical decision experiments document for morphologically complex words two points at which the probability of a target word given the evidence shifts dramatically. The first point is reached when morphologically unrelated competitors are no longer compatible with the evidence. Adapting terminology from Marslen-Wilson (1984), we refer to this as the word's initial uniqueness point (UP1). The second point is the complex uniqueness point (CUP) introduced by Balling and Baayen (2008), at which morphologically related competitors become incompatible with the input. Later initial as well as complex uniqueness points predict longer response latencies. We argue that the effects of these uniqueness points arise due to the large surprisal (Levy, 2008) carried by the phonemes at these uniqueness points, and provide independent evidence that how cumulative surprisal builds up in the course of the word co-determines response latencies. The presence of effects of surprisal, both at the initial uniqueness point of complex words, and cumulatively throughout the word, challenges the Shortlist B model of Norris and McQueen (2008), and suggests that a Bayesian approach to auditory comprehension requires complementation from information theory in order to do justice to the cognitive cost of updating probability distributions over lexical candidates. Copyright © 2012 Elsevier B.V. All rights reserved.

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48 CFR 25.700 - Scope of subpart.

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Two Thai families with Norrie disease (ND): association of two novel missense mutations with severe ND phenotype, seizures, and a manifesting carrier.

PubMed

Yamada, K; Limprasert, P; Ratanasukon, M; Tengtrisorn, S; Yingchareonpukdee, J; Vasiknanonte, P; Kitaoka, T; Ghadami, M; Niikawa, N; Kishino, T

2001-04-15

We describe two Thai families with Norrie disease (ND) in three generations, including 10 affected males and one manifesting female. All affected males in each family had severely defective eye development with complete loss of vision. In addition, three male patients (one from family 1 and two from family 2) suffered from epilepsy, and one female carrier from one family manifested blindness with phthisis bulbi in her right eye. Mutation analysis of the ND gene (NDP) revealed two different novel missense mutations (L16P and S75P) that co-segregated with ND in each family, suggesting that the newly appearing proline at codon 16 or codon 75 alters the conformation of the ND protein and contributes to the severe phenotype of ND in each family. Other studies suggest that epileptic seizures or growth retardation that is associated with ND is the consequence of loss of contiguous genes, because most such patients had deletions extending beyond the Norrie locus. Our finding that the three affected males in the two families with the missense mutations had epilepsy does not support a contiguous gene effect, but favors the pleiotropism of NDP, at least as far as the epileptic manifestation is concerned. The unilateral blindness in the female carrier may have been due to non-random X-inactivation. Copyright 2001 Wiley-Liss, Inc.

Factors related to respiration influencing survival and respiratory function in patients with amyotrophic lateral sclerosis: a retrospective study.

PubMed

Leonardis, L; Dolenc Grošelj, L; Vidmar, G

2012-12-01

Various breathing abnormalities (Neurology 2009; 73: 1218) have been proposed as indicators for the introduction of non-invasive positive-pressure ventilation (NIV) in patients with amyotrophic lateral sclerosis (ALS). We were interested in the usefulness of symptoms of respiratory insufficiency and abnormal results of daytime arterial gas analyses (AGA) as predictors of survival and the effect of NIV on respiratory volumes and pressures. Reported symptoms, respiratory subscore of the ALS Functional Rating Scale (ALSFRS-r), Norris scale (Norris-r), and AGA were retrospectively analyzed in 189 ALS patients. Longitudinal follow-up of forced vital capacity (FVC), maximal inspiratory and expiratory pressure (MIP, MEP), and sniff nasal pressure (SNP) were analyzed with regard to the introduction of NIV. Respiratory symptoms were a bad prognostic sign (P = 0.007). Abnormalities in Norris-r, ALSFRS-r, pO(2), pCO(2), and oxygen saturation tended to be associated with a shorter survival, although they were not statistically significant. NIV prolonged survival and reduced the decline in FVC (P = 0.007), MIP, MEP, and SNP (the last three were not statistically significant). Symptoms, abnormal FVC, and AGA do not always coincide, and they can appear in a different sequence. Any respiratory abnormality should prompt the clinician to start discussing NIV with the patient. NIV prolongs survival and improves respiratory function. © 2012 The Author(s) European Journal of Neurology © 2012 EFNS.

The importance of biochemical and genetic findings in the diagnosis of atypical Norrie disease.

PubMed

Rodríguez-Muñoz, Ana; García-García, Gema; Menor, Francisco; Millán, José M; Tomás-Vila, Miguel; Jaijo, Teresa

2018-01-26

Norrie disease (ND) is a rare X-linked disorder characterized by bilateral congenital blindness. ND is caused by a mutation in the Norrie disease pseudoglioma (NDP) gene, which encodes a 133-amino acid protein called norrin. Intragenic deletions including NDP and adjacent genes have been identified in ND patients with a more severe neurologic phenotype. We report the biochemical, molecular, clinical and radiological features of two unrelated affected males with a deletion including NDP and MAO genes. Biochemical and genetic analyses were performed to understand the atypical phenotype and radiological findings. Biogenic amines in cerebrospinal fluid (CSF) were measured by high-performance liquid chromatography. The coding exons of NDP gene were amplified by polymerase chain reaction. Multiplex ligation-dependent probe amplification and chromosomal microarray were carried out on both affected males. Computed tomography and magnetic resonance imaging were performed on the two patients. In one patient, the serotonin and catecholamine metabolite levels in CSF were virtually undetectable. In both patients, genetic studies revealed microdeletions in the Xp11.3 region, involving the NDP, MAOA and MAOB genes. Radiological examination demonstrated brain and cerebellar atrophy. We suggest that alterations caused by MAO deficit may remain during the first years of life. Clinical phenotype, biochemical findings and neuroimaging can guide the genetic study in patients with atypical ND and help us to a better understanding of this disease.

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..., Compensation, and Liability Act, 42 U.S.C. 9603(a) (``CERCLA''), the United States sought penalties and... defendant's train in Graniteville, South Carolina, which resulted in the death of nine people, evacuation of the surrounding community, and environmental injury including the death of hundreds of fish in nearby...

X-linked juvenile retinoschisis: mutations at the retinoschisis and Norrie disease gene loci?

PubMed

Hiraoka, M; Rossi, F; Trese, M T; Shastry, B S

2001-01-01

Juvenile retinoschisis (RS) and Norrie disease (ND) are X-linked recessive retinal disorders. Both disorders, in the majority of cases, are monogenic and are caused by mutations in the RS and ND genes, respectively. Here we report the identification of a family in which mutations in both the RS and ND genes are segregating with RS pathology. Although the mutations identified in this report were not functionally characterized with regard to their pathogenicity, it is likely that both of them are involved in RS pathology in the family analyzed. This suggests the complexity and digenic nature of monogenic human disorders in some cases. If this proves to be a widespread problem, it will complicate the strategies used to identify the genes involved in diseases and to develop methods for intervention.

Submicroscopic interstitial deletion of the X chromosome explains a complex genetic syndrome dominated by Norrie disease.

PubMed

Gal, A; Wieringa, B; Smeets, D F; Bleeker-Wagemakers, L; Ropers, H H

1986-01-01

Norrie disease (ND), an X-linked recessive disorder, is characterized by congenital blindness followed by bulbar atrophy. We have examined a three-generation family in which ND is part of a complex X-linked syndrome with severe mental retardation, hypogonadism, growth disturbances, and increased susceptibility to infections as additional features. This syndrome is apparently due to an interstitial deletion, as evidenced by the failure of the L1.28 DNA probe (DXS7 locus, Xp11.3) to detect complementary DNA sequences on the defective X chromosome of an affected male and of several obligatory heterozygotes. Attempts to further define this deletion with other DNA probes from the proximal short arm of the X chromosome or by prometaphase chromosome analysis were unsuccessful.

1. Photocopy of 1910 photograph in Louis Sanchez Collection, Oakland, ...

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

1. Photocopy of 1910 photograph in Louis Sanchez Collection, Oakland, California. Rephotographed August 1940. VIEW OF FRONT - Frank Norris Memorial, Redwood Retreat Road, Gilroy, Santa Clara County, CA

Real-time Remote Data Online For Norris Geyser Basin in Yellowstone National Park

NASA Astrophysics Data System (ADS)

Perry, J. E.; Lowenstern, J. B.; Clor, L.; Cervelli, P. F.; Allen, S. T.; Heasler, H.; Moloney, T.

2010-12-01

Perry, John, Lowenstern, Jacob, Cervelli, Peter, Clor, Laura, Heasler, Henry, Allen, Scott, Moloney, Tim During June 2010, ten 900MHz wireless temperature data loggers (nodes) were installed around the Norris Geyser Basin to monitor geothermal features, streams and soil temperatures. The loggers can provide near real-time updates on temperature variations within 0.2 deg C due to hydrothermal discharges or subsurface fluid migration. Each sensor node is programmed to measure the temperature every two minutes and automatically upload data to the base station computer daily. The hardware consists of a waterproof case containing an M5 logger (made by Marathon Products, Inc.®) with internal memory, lithium D-cell batteries and a 900 MHz, 1-W-transceiver and 5 meter long Teflon-coated probe with a thermistor sensor. Tethered stub or panel antennas are oriented to optimize signal strength to the base station near the Norris Museum. A 0.61 meter-long base-station antenna located 10m high provides signal to the furthest node over 850 meters away with most being “line-of-sight”. A 20-meter coaxial cable and lightning grounding wire connects the base-station antenna to an Ethernet-radio connected to the YNP local-area network. A server located 26-km north at Mammoth Hot Springs requests data at regular intervals (normally daily), archives the information, and then sends it to the USGS for further archiving and internet distribution. During periods of unusual hydrothermal behavior, data can be requested as needed, and it is possible to set user-programmable alarm limits for notification. The RF network is designed to monitor changes from three different sub-basins at Norris (Gray Lakes, Steamboat-Echinus and Porcelain Basin), the main Tantalus Creek drainage, and five individual thermal features (Constant, Porkchop, Steamboat and Echinus Geysers, and Opalescent Spring). The logger installed in Nuphar Lake provides ambient temperatures controlled solely by local meteorological conditions. Soil temperature is measured near Vixen Geyser. The system will permit land managers, scientists and the public to keep track of changes to the hydrothermal system, including geyser eruptions, periodic basin-wide disturbances, or fluid-release events that may accompany or follow seismic activity.

Preparing medical students to care for older adults: the impact of a Senior Mentor Program.

PubMed

Eleazer, G Paul; Wieland, Darryl; Roberts, Ellen; Richeson, Nancy; Thornhill, Joshua T

2006-04-01

To provide alternative training experiences for medical students to improve the competencies needed to provide care for older adults. Part of a comprehensive approach to integrating geriatric content at the University of South Carolina (USC) School of Medicine, the Senior Mentor Program (SMP) was launched in 2000. The SMP links pairs of students with older community volunteers in the spring of the first year. Students visit their mentors throughout medical school, and execute assignments that complement materials covered in the traditional curriculum. To evaluate the feasibility and efficacy of the SMP, the authors describe the program's operation and outputs, the extent to which undergraduate medical education at USC and the SMP meet the core competencies for care of older adults promulgated by the American Geriatrics Society (AGS), and analyze the coverage of geriatrics content from 2000 to 2005 at USC, as indicated by responses to the Medical School Graduation Questionnaire. From 2000 to 2005, 379 students enrolled in the SMP; 133 graduates of the classes of 2004 and 2005 have completed the SMP. Students and mentors indicated high acceptance and enthusiasm. The program has been maintained with minimal administrative burden. USC covered only half of AGS competencies before the SMP, but now covers 100%, with nearly a third met specifically through the SMP. USC graduates reporting inclusion of geriatrics throughout their four years of training rose from 66% in 2002 to 96% in 2004-05. The SMP has had a substantial impact on students' preparation for dealing with an aging patient population.

A New Look at Contextualization and Application: A Comprehensive Pedagogical Strategy for Business German.

ERIC Educational Resources Information Center

Strack, Thomas; Clausing, Gerhard

This paper discusses the problems faced by teachers of business German, the possible goals of business German courses, and the design of an effective textbook to integrate business content and language skills. Special reference is made to the two-semester business German course offered by the University of Southern California (USC). The major goal…

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... (``CERCLA''), 42 U.S.C. 9607(a)(3), for response costs incurred by the Environmental Protection Agency..., Inc. associated with costs incurred by EPA at the Pioneer Smelting Superfund Site. The Department of... (25 cents per page reproduction cost) payable to the U.S. Treasury or, if by e-mail or fax, forward a...

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Tea Tree Oil

MedlinePlus

... oil without problems, but some people may develop contact dermatitis (an allergic skin rash) or skin irritation on ... References Jack AR, Norris PL, Storrs FJ. Allergic contact dermatitis to plant extracts in cosmetics . Seminars in Cutaneous ...

Mutations of the Norrie gene in Korean ROP infants.

PubMed

Kim, Jeong Hun; Yu, Young Suk; Kim, Jiyeon; Park, Seong Sup

2002-12-01

The present study was conducted to evaluate if there is a Norrie disease gene (ND gene) mutation involved in the retinopathy of prematurity (ROP), and to identify the possibility of a genetic abnormality that may be linked to the presence of ROP. Nineteen premature Korean infants, with a low birth weight (1500 g or less) or low gestational age (32 weeks or less), were included in the study. Eighteen infants had ROP, and the other did not. Genomic DNA was isolated from the peripheral blood leukocytes of these patients, and all three exons and their flanking areas, all known ND gene mutations regions, were evaluated following amplification by a polymerase chain reaction, but no ND gene mutations were detected. Any disagreement between the relationship of ROP to the ND gene mutation will need to be clarified by further investigation.

KENNEDY SPACE CENTER, FLA. - Amy Norris (left) packs some of the debris stored in the Columbia Debris Hangar. Jack Nowling works on the computer. About 83,000 pieces of debris were shipped to KSC during search and recovery efforts in East Texas. That represents about 38 percent of the dry weight of Columbia, equaling almost 85,000 pounds. An area of the Vehicle Assembly Building is being prepared to store the debris.

NASA Image and Video Library

2003-09-10

KENNEDY SPACE CENTER, FLA. - Amy Norris (left) packs some of the debris stored in the Columbia Debris Hangar. Jack Nowling works on the computer. About 83,000 pieces of debris were shipped to KSC during search and recovery efforts in East Texas. That represents about 38 percent of the dry weight of Columbia, equaling almost 85,000 pounds. An area of the Vehicle Assembly Building is being prepared to store the debris.

A novel contiguous deletion involving NDP, MAOB and EFHC2 gene in a patient with familial Norrie disease: bilateral blindness and leucocoria without other deficits.

PubMed

Jia, Bei; Huang, Liping; Chen, Yaoyu; Liu, Siping; Chen, Cuihua; Xiong, Ke; Song, Lanlin; Zhou, Yulai; Yang, Xinping; Zhong, Mei

2017-12-01

Contiguous microdeletions of the Norrie disease pseudoglioma (NDP) region on chromosome Xp11.3 have been widely confirmed as contributing to the typical clinical features of Norrie disease (ND). However, the precise relation between genotype and phenotype could vary. The contiguous deletion of NDP and its neighbouring genes, MAOA/B and EFHC2, reportedly leads to syndromic clinical features such as microcephaly, intellectual disability, and epilepsy. Herewe report a novel contiguous microdeletion of the NDP region containing the MAOB and EFHC2 genes,which causes eye defects but no cognitive disability.We detected a deletion of 494.6 kb atXp11.3 in both the proband and carrier mother. This deletionwas then used as the molecular marker in prenatal diagnosis for two subsequent pregnancies. The deletion was absent in one of the foetuses, who remain without any abnormalities at 2 years of age. The proband shows the typical ocular clinical features of ND including bilateral retinal detachment, microphthalmia, atrophic irides, corneal opacification, and cataracts, but no symptoms of microcephaly, intellectual disability, and epilepsy. This familial study demonstrates that a deficiency in one of two MAO genes may not lead to psychomotor delay, and deletion of EFHC2 may not cause epilepsy. Our observations provide new information on the genotype-phenotype relations of MAOA/B and EFHC2 genes involved in the contiguous deletions of ND.

Unilateral sporadic retinal dysplasia: Results of histopathologic, immunohistochemical, chromosomal, genetic, and VEGF-A analyses

PubMed Central

Jakobiec, Frederick A.; Zakka, Fouad R.; D’Amato, Robert; DeAngelis, Margaret M.; Walton, David S.; Rao, Rajesh C.

2014-01-01

PURPOSE To describe new findings in a case of unilateral retinal dysplasia. METHODS Histopathologic evaluation of an enucleated globe and analysis with immunohistochemical probes, karyotyping, and genetic analysis for the Norrie gene, and aqueous assay for vascular endothelial growth factor A (VEGF-A). RESULTS Histopathological examination of the globe revealed retinal dysplasia with pseudorosette formation, abnormal or absent retinal nuclear lamination, a paucity of disorganized retinal microvasculature, retinal infoldings, advanced gliosis, persistent hyperplastic vitreous, exuberant neovascularization of the vitreous, and iris neovascularization (identical to the findings observed in bilateral Norrie disease). Immunohistochemistry disclosed GFAP-positive and GLUT-1-positive gliosis and retinal and persistent hyperplastic vitreous microvessels that were CD34-positive and GLUT-1-negative. Ki-67-positive retinal cells were polarized toward the subretinal space and absent in the retinal invaginations and pseudorosettes. A normal karyotype was found, and DNA sequencing revealed no known mutation in the region of the Norrie gene (NDP) in sputum or retinal DNA. Aqueous obtained immediately after enucleation contained an exceptionally high concentration of VEGF-A (4.5 ng/mL). CONCLUSIONS Despite the failure to find an abnormal NDP allele, other unexplored NDP regions, an undetected defect restricted to retinal tissues, or an autosomal mutation coupled with disrupted signaling pathways may be responsible for the condition. High aqueous VEGF-A suggests that this cytokine may play a role in pathogenesis in conjunction with other pathways. PMID:22153404

Is a More Comprehensive Surgery Necessary in Patients With Uterine Serous Carcinoma?

PubMed

Touhami, Omar; Trinh, Xuan-Bich; Gregoire, Jean; Sebastianelli, Alexandra; Renaud, Marie-Claude; Grondin, Katherine; Plante, Marie

2015-09-01

Uterine serous carcinoma (USC) is an aggressive histologic subtype of endometrial cancer that shares similarities to serous ovarian cancer, with a propensity for spread to the upper abdomen, a high recurrence rate, and a poor prognosis. The aim of this study was to determine whether the traditional surgical staging procedure for endometrial cancer was adequate for USC or whether a more extensive surgery, similar to the staging procedure for ovarian cancer, needs to be performed. Specifically, the roles of omentectomy and sentinel lymph node (SLN) mapping were evaluated. We retrospectively identified cases of presumed clinical stage I USC at our institution from April 2005 to March 2014. Medical records were reviewed for the following information: age at diagnosis, preoperative imaging, operative findings, surgical procedure, and final histology with definitive International Federation of Gynecology and Obstetrics stage. A total of 39 patients with presumed clinical stage I USC were identified. According to the final pathology report, the surgical stage was as follows: 17 stage IA (44%), 8 stage IB (20%), 3 stage II (8%), 2 stage IIIA (5%), 6 stage IIIC1 (15%), 1 IIIC2 (3%), and 2 stage IVB (5%). Therefore, 14 patients (36%) were surgically upstaged, but none of the patients had their clinical disease upstaged by virtue of finding microscopic metastatic disease in an otherwise normal-looking omentum. Sentinel lymph node mapping was performed in 19 patients (42%). Sensitivity and negative predictive value of SLN mapping were 100% when at least 1 SLN was identified. The detection of microscopic disease in radiologically and clinically normal-appearing omentum seems to be rare in USC. Sentinel lymph node mapping seems to be valuable in the serous subtype of endometrial cancer. A less extensive surgery may be possible in patients with USC as it seems to provide the same information as a more extensive surgery.

75 FR 30859 - Notice of Lodging of Consent Decree Under the Comprehensive Environmental Response, Compensation...

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..., Compensation and Liability Act of 1980, 42 U.S.C. 9601-9675, for recovery of costs incurred by the United... of costs incurred by EPA at the Site. The Department of Justice will receive for a period of thirty... Library, please enclose a check in the amount of $23.25 (25 cents per page reproduction cost) payable to...

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... sections 172(c)(9) and 182(c)(9). \\1\\ Our Clean Data Policy is set forth in a May 10, 1995 EPA memorandum... and proposed on January 19, 2010 (75 FR 2938). We expect by August 2010 to have completed our... without going through the comprehensive redesignation procedures of 42 U.S.C. 7407(d)(3). Response: This...

76 FR 70544 - OFAC Implementation of Certain Sanctions Imposed on Seven Persons by the Secretary of State...

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2011-11-14

... Sanctions Imposed on Seven Persons by the Secretary of State Pursuant to the Iran Sanctions Act of 1996, as... sanctions imposed on seven persons by the Secretary of State pursuant to the Iran Sanctions Act of 1996 (Pub. L. 104-172) (50 U.S.C. 1701 note) (``ISA''), as amended by the Comprehensive Iran Sanctions...

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... Sanctions Imposed on SYTROL by the Secretary of State Pursuant to the Iran Sanctions Act of 1996, as Amended... sanctions imposed on SYTROL by the Secretary of State pursuant to the Iran Sanctions Act of 1996 (Pub. L. 104-172) (50 U.S.C. 1701 note) (``ISA''), as amended by the Comprehensive Iran Sanctions...

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Molecular alterations of EGFR and PIK3CA in uterine serous carcinoma.

PubMed

Hayes, Monica Prasad; Douglas, Wayne; Ellenson, Lora Hedrick

2009-06-01

Uterine serous carcinoma (USC) is an aggressive endometrial cancer associated with poor prognosis despite comprehensive surgical staging and adjuvant chemotherapy and radiation therapy. Biologic targets have yet to be fully explored in this disease and research on such targets could lead to clinical trials utilizing a new class of therapeutics. This study sought to evaluate primary USC tumors for molecular alterations in epidermal growth factor receptor (EGFR) and the recently characterized oncogene PIK3CA, which encodes the catalytic p110-alpha subunit of phosphatidylinositol 3-kinase (PI3K) and thus activates the AKT-mTOR oncogenic pathway. Paraffin-embedded archival tissue of 45 primary USC tumors was utilized in this study. Immunohistochemical analysis of EGFR was performed and cases given a score of 0 to 12 calculated as the product of staining intensity (0 to 3+) and the percentage of positively stained cells (0-4), with 1=1-25%, 2=26-50%, 3=51-75%, and 4=76-100%. For mutational analysis, neoplastic tissue was microdissected and DNA was extracted with phenol-chloroform. Exons 18 through 21 of EGFR and exons 9 and 20 of PIK3CA, the most commonly mutated exons of these genes, were amplified and directly sequenced. When EGFR was evaluated, moderate or strong EGFR membranous staining was observed in 25/45 (56%) USC cases. Thus, a mutational analysis was performed on 35 cases, including all cases with moderate and strong EGFR staining. No mutations were identified in EGFR. In contrast, PIK3CA mutations were confirmed in 5/34 (15%) of USC cases. Four cases were mutated in exon 20 and one case was mutated in exon 9. Since optimal treatment of uterine serous carcinoma remains unknown, novel therapeutic approaches need to be actively pursued. In the current study of primary USC tumors, oncogenic mutations of the PIK3CA gene were seen in 15% of USC cases. This represents the first report of this gene mutation in USC. In addition, EGFR stained positively in the majority of cases, suggesting a possible target protein. These findings warrant further investigation and suggest a potential role for therapeutic agents targeting the PI3K-AKT-mTOR pathway, such as rapamycin, as well as possible targets of EGFR in the treatment of uterine serous carcinoma.

Contractor cuts solar gain with custom window curtain

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

Ingraham, R.

1985-04-22

A local energy services contractor, using a 210-foot motorized thermal curtain to deflect sunlight entering the windows, has reduced excessive heat gain at a racetrack clubhouse here, cutting air conditioning run-time by about 75%. Because Mobile Greyhound Park's clubhouse faces east, direct exposure to sunlight from dawn to afternoon race time, about 1 p.m., was bringing the indoor temperature to almost 100/sup 0/, according to Stan Norris, operations management for the contracting firm, Technical Energy Controls, Inc. Lowering the temperature to a comfortable level required running four Carrier Corporation air handling units, rated at a total of more than 170more » tons, for about eight hours, and annual energy bills were running at about $240,000, Norris said. With the aluminum foil-backed vinyl curtain, the air handling units need only operate two hours to bring temperatures to comfort levels.« less

Novel mutation at the initiation codon in the Norrie disease gene in two Japanese families.

PubMed

Isashiki, Y; Ohba, N; Yanagita, T; Hokita, N; Doi, N; Nakagawa, M; Ozawa, M; Kuroda, N

1995-01-01

We have identified a new mutation of Norrie disease (ND) gene in two Japanese males from unrelated families; they showed typical ocular features of ND but no mental retardation or hearing impairment. A mutation was found in both patients at the initiation codon of exon 2 of the ND gene (ATG to GTG), with otherwise normal nucleotide sequences. Their mothers had the normal and mutant types of the gene, which was expected for heterozygotes of the disease. The mutation of the initiation codon would cause the failure of ND gene expression or a defect in translation thereby truncating the amino terminus of ND protein. In view of the rarity and marked heterogeneity of mutations in the ND gene, the present apparently unrelated Japanese families who have lived in the same area for over two centuries presumably share the origin of the mutation.

Supra-subduction zone extensional magmatism in Vermont and adjacent Quebec: Implications for early Paleozoic Appalachian tectonics

USGS Publications Warehouse

Kim, J.; Coish, R.; Evans, M.; Dick, G.

2003-01-01

Metadiabasic intrusions of the Mount Norris Intrusive Suite occur in fault-bounded lithotectonic packages containing Stowe, Moretown, and Cram Hill Formation lithologies in the northern Vermont Rowe-Hawley belt, a proposed Ordovician arc-trench gap above an east-dipping subduction zone. Rocks of the Mount Norris Intrusive Suite are characteristically massive and weakly foliated, have chilled margins, contain xenoliths, and have sharp contacts that both crosscut and are parallel to early structural fabrics in the host metasedimentary rocks. Although the mineral assemblage of the Mount Norris Intrusive Suite is albite + actinolite + epidote + chlorite + calcite + quartz, intergrowths of albite + actinolite are probably pseudomorphs after plagioclase + clinopyroxene. The metadiabases are subalkaline, tholeiitic, hypabyssal basalts with preserved ophitic texture. A backarc-basin tectonic setting for the intrusive suite is suggested by its LREE (light rare earth element) enrichment, negative Nb-Ta anomalies, and Ta/Yb vs. Th/Yb trends. Although no direct isotopic age data are available, the intrusions are broadly Ordovician because their contacts are clearly folded by the earliest Acadian (Silurian-Devonian) folds. Field evidence and geochemical data suggest compelling along-strike correlations with the Coburn Hill Volcanics of northern Vermont and the Bolton Igneous Group of southern Quebec. Isotopic and stratigraphic age constraints for the Bolton Igneous Group bracket these backarc magmas to the 477-458 Ma interval. A tectonic model that begins with east-dipping subduction and progresses to outboard west-dipping subduction after a syncollisional polarity reversal best explains the intrusion of deformed metamorphosed metasedimentary rocks by backarc magmas.

Vascular changes in the cerebellum of Norrin /Ndph knockout mice correlate with high expression of Norrin and Frizzled-4.

PubMed

Luhmann, Ulrich F O; Neidhardt, John; Kloeckener-Gruissem, Barbara; Schäfer, Nikolaus F; Glaus, Esther; Feil, Silke; Berger, Wolfgang

2008-05-01

X-linked Norrie disease, familial exudative vitreoretinopathy (FEVR), Coat's disease and retinopathy of prematurity are severe human eye diseases and can all be caused by mutations in the Norrie disease pseudoglioma gene. They all show vascular defects and characteristic features of retinal hypoxia. Only Norrie disease displays additional neurological symptoms, which are sensorineural hearing loss and mental retardation. In the present study, we analysed transcript levels of the ligand Norrin (Ndph) and its two receptors Frizzled-4 (Fzd4) and LDL-related protein receptor 5 (Lrp5) in six different brain regions (cerebellum, cortex, hippocampus, olfactory bulb, pituitary and brain stem) of 6- to 8-month-old wild-type and Ndph knockout mice by quantitative real-time PCR. No effect of the Ndph knockout allele on Fzd4 or Lrp5 receptor expression was found. Furthermore, no alterations of the transcript levels of three hypoxia-regulated angiogenic factors (Vegfa, Itgrb3 and Tie1) were observed in the absence of Norrin. Interestingly, we identified significant differences in Ndph, Fzd4 and Lrp5 transcript levels in brain regions of wild-type mice and observed highest expression of Norrin and frizzled-4 in cerebellum. Transcript analyses were correlated with morphological data obtained from cerebellum and immunohistochemical studies of blood vessels in different brain regions. Vessel density was reduced in the cerebellum of Ndph knockout mice but the number of Purkinje and granular cells was not altered. This provides the first description of a brain phenotype in Ndph knockout mice, which will help to elucidate the role of Norrin in the brain.

75 FR 62858 - Notice of Lodging of Consent Decree Under Sections 107(A) and 113(G)(2) of The Comprehensive...

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Genetics Home Reference: Norrie disease

MedlinePlus

... is an inherited eye disorder that leads to blindness in male infants at birth or soon after birth. It causes abnormal development of the retina , the layer of sensory cells that detect light and color, with masses of immature retinal cells accumulating at ...

EXTERIOR ELEVATION, LOOKING SOUTH, ALSO SHOWING THE NORFOLK SOUTHERN STEAM ...

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

EXTERIOR ELEVATION, LOOKING SOUTH, ALSO SHOWING THE NORFOLK SOUTHERN STEAM RESTORATION FLOOR INTERIOR WITH A DRILL PRESS (LEFT) AND BORING MILL (RIGHT). - Norfolk & Southern Steam Locomotive No. 1218, Norris Yards, East of Ruffner Road, Irondale, Jefferson County, AL

77 FR 68152 - NASA Advisory Council; Science Committee; Meeting

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... NATIONAL AERONAUTICS AND SPACE ADMINISTRATION [Notice (12-086)] NASA Advisory Council; Science..., the National Aeronautics and Space Administration (NASA) announces a meeting of the Science Committee.... Marian Norris, Science Mission Directorate, NASA Headquarters, Washington, DC 20546, (202) 358-4452, fax...

Having a usual source of care and its associated factors in Korean adults: a cross-sectional study of the 2012 Korea Health Panel Survey.

PubMed

An, Ah Reum; Kim, Kyoungwoo; Lee, Jae-Ho; Sung, Nak-Jin; Lee, Sang-Il; Hyun, Min Kyung

2016-11-29

Usual source of care (USC) is one of the hallmarks of primary care. We aimed to examine the status of having a USC and its patient-related sociodemographic factors among Korean adults. Data were obtained from the 2012 Korea Health Panel survey. Panel participants were selected for the study who were aged 18 years or older and who replied to questionnaire items on having a USC (n = 11,935). Of the participants, 21.5% had a usual place and 13.9% had a usual physician. Reasons for not having a USC were seldom being ill (66.1%), the preference to visit multiple medical institutions (27.9%), and others. The private community clinic was the most common type of usual place (57.0%). In patient-reported attributes of care provided by a usual physician, the percentages of positive responses for comprehensiveness and coordination were 67.2% and 34.5%, respectively. By institution type, primary care clinics showed the lowest percentage (32.8%) of positive responses for coordination. Adjusted odds ratios of having a usual physician were 3.77 (95% confidence interval, CI: 3.75-3.79) for those aged 65 years or older (vs. aged 18-34 years), 1.31 (CI: 1.30-1.31) for females (vs. males), 0.72 (CI: 0.72-0.73) for unmarried people (vs. married), 1.16 (CI: 1.16-1.16) for college graduates or higher (vs. elementary school graduate or less), 0.64 for the fifth quintile (vs. the first quintile) by household income, 1.53 (CI: 1.52-1.54) for Medical Aid (vs. employee health insurance) for type of health insurance, and 4.09 (CI: 4.08-4.10) for presence (vs. absence) of a chronic diseases. The proportion of Korean adults who have a USC is extremely low, the most influential factor of having a USC is having a chronic disease or not, and Korean patients experience much poorer health care coordination than do patients in other industrialized countries. The findings of this study will give insight to researchers and policy makers regarding the potential facilitators of and barriers to promoting having a USC in the general Korean public.

The Nicaragua Canal:Security And Economic Boom Or Bust

DTIC Science & Technology

2014-04-01

Advisor: Dr. Kathleen Mahoney -Norris Maxwell Air Force Base, Alabama April 2014 DISTRIBUTION A. Approved for public release: Distribution...Commerce and the Future of the Panama Canal. Cambridge, MD: Cornell Maritime Press, Inc., 1975 . Panama Canal Authority. “History.” https

Evaluation of the evolving stress field of the Yellowstone volcanic plateau, 1988 to 2010, from earthquake first-motion inversions

NASA Astrophysics Data System (ADS)

Russo, E.; Waite, G. P.; Tibaldi, A.

2017-03-01

Although the last rhyolite eruption occurred around 70 ka ago, the silicic Yellowstone volcanic field is still considered active due to high hydrothermal and seismic activity and possible recent magma intrusions. Geodetic measurements document complex deformation patterns in crustal strain and seismic activity likewise reveal spatial and temporal variations in the stress field. We use earthquake data recorded between 1988 and 2010 to investigate these variations and their possible causes in more detail. Earthquake relocations and a set of 369 well-constrained, double-couple, focal mechanism solutions were computed. Events were grouped according to location and time to investigate trends in faulting. The majority of the events have normal-faulting solutions, subordinate strike-slip kinematics, and very rarely, reverse motions. The dominant direction of extension throughout the 0.64 Ma Yellowstone caldera is nearly ENE, consistent with the perpendicular direction of alignments of volcanic vents within the caldera, but our study also reveals spatial and temporal variations. Stress-field solutions for different areas and time periods were calculated from earthquake focal mechanism inversion. A well-resolved rotation of σ3 was found, from NNE-SSW near the Hebgen Lake fault zone, to ENE-WSW near Norris Junction. In particular, the σ3 direction changed throughout the years around Norris Geyser Basin, from being ENE-WSW, as calculated in the study by Waite and Smith (2004), to NNE-SSW, while the other σ3 directions are mostly unchanged over time. The presence of ;chocolate tablet; structures, with two sets of nearly perpendicular normal faults, was identified in many stages of the deformation history both in the Norris Geyser Basin area and inside the caldera.

THE USE OF TRACI FOR POLLUTION PREVENTION AND SUSTAINABLE DEVELOPMENT

EPA Science Inventory

The Use of TRACI for
Pollution Prevention and Sustainable Development

Jane C. Bare1 and Gregory A. Norris2
1) Systems Analysis Branch, Sustainable Technology Division, National Risk Management Research Laboratory, U.S. Environmental Protection Agency, Cincinnati, O...

78 FR 20358 - NASA Advisory Council; Science Committee; Heliophysics Subcommittee; Meeting

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2013-04-04

... NATIONAL AERONAUTICS AND SPACE ADMINISTRATION [Notice: 13-036] NASA Advisory Council; Science... Subcommittee (HPS) of the NASA Advisory Council (NAC). This Subcommittee reports to the Science Committee of... CONTACT: Ms. Marian Norris, Science Mission Directorate, NASA Headquarters, Washington, DC 20546, (202...

77 FR 21756 - Agency Information Collection Extension

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2012-04-11

... Norris at U.S. Energy Information Administration, Office of Petroleum and Biofuels Statistics, U.S... 1995. Weekly petroleum and biofuels supply surveys (Forms EIA-800, 802, 803, 804, 805, and 809) are used to gather data on petroleum refinery operations, blending, biofuels production, inventory levels...

75 FR 61530 - Issuance of Regulatory Guides

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2010-10-05

... Materials Code Case Acceptability, ASME Section III,'' and RG 1.147, Rev. 16, ``Inservice Inspection Code Case Acceptability, ASME Section XI, Division 1.'' FOR FURTHER INFORMATION CONTACT: Wallace E. Norris... specific problems or postulated accidents, and data the staff needs in its review of applications for...

75 FR 2893 - NASA Advisory Council; Science Committee; Astrophysics Subcommittee; Meeting

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2010-01-19

... Science Committee of the NAC. The Meeting will be held for the purpose of soliciting from the scientific... NATIONAL AERONAUTICS AND SPACE ADMINISTRATION [Notice (10-002)] NASA Advisory Council; Science... FURTHER INFORMATION CONTACT: Ms. Marian Norris, Science Mission Directorate, NASA Headquarters, Washington...

75 FR 13597 - NASA Advisory Council; Science Committee; Astrophysics Subcommittee; Meeting

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2010-03-22

... Science Committee of the NAC. The Meeting will be held for the purpose of soliciting from the scientific... NATIONAL AERONAUTICS AND SPACE ADMINISTRATION [Notice (10-031)] NASA Advisory Council; Science.... FOR FURTHER INFORMATION CONTACT: Ms. Marian Norris, Science Mission Directorate, NASA Headquarters...

Teaching Reprint File Management: Basic Principles and Software Programs.

ERIC Educational Resources Information Center

Wood, Elizabeth H.

1989-01-01

Describes a workshop for teaching library users how to manage reprint files which was developed at the University of Southern California Norris Medical Library. Software programs designed for this purpose are suggested, and a sidebar lists software features to consider. (eight references) (MES)

76 FR 10940 - Notice of Delays in Processing of Special Permits Applications

Federal Register 2010, 2011, 2012, 2013, 2014

2011-02-28

...-M Phoenix Air Group, Inc., 4 03-31-2011 Cartersville, GA. 10869-M Norris Cylinder Company, 4 03-31........ 4 01-31-2011 10656-M The Conference of Radiation 4 04-30-2011 Control Program Directors, Inc., Frankfort, KY. [[Page 10941

Wnt signaling: Ig-norrin the dogma.

PubMed

Clevers, Hans

2004-06-08

Secreted Wnt proteins trigger the intracellular Wnt signaling cascade upon engagement of dedicated Frizzled-Lrp receptor complexes. Unexpectedly, a non-Wnt ligand for this receptor complex has now been discovered. This novel ligand, Norrin, is mutated in the hereditary ocular Norrie syndrome. Copyright 2004 Elsevier Ltd.

A novel missense mutation in the NDP gene in a child with Norrie disease and severe neurological involvement including infantile spasms.

PubMed

Lev, Dorit; Weigl, Yuval; Hasan, Mariana; Gak, Eva; Davidovich, Michael; Vinkler, Chana; Leshinsky-Silver, Esther; Lerman-Sagie, Tally; Watemberg, Nathan

2007-05-01

Norrie disease (ND) is a rare X-linked recessive disorder characterized by congenital blindness and in some cases, mental retardation and deafness. Other neurological complications, particularly epilepsy, are rare. We report on a novel mutation identified in a patient with ND and profound mental retardation. The patient was diagnosed at the age of 6 months due to congenital blindness. At the age of 8 months he developed infantile spasms, which were diagnosed at 11 months as his EEG demonstrated hypsarrhythmia. Mutation analysis of the ND gene (NDP) of the affected child and his mother revealed a novel missense mutation at position c.134T > A resulting in amino acid change at codon V45E. To the best of our knowledge, such severe neurological involvement has not been previously reported in ND patients. The severity of the phenotype may suggest the functional importance of this site of the NDP gene.

An animal model for Norrie disease (ND): gene targeting of the mouse ND gene.

PubMed

Berger, W; van de Pol, D; Bächner, D; Oerlemans, F; Winkens, H; Hameister, H; Wieringa, B; Hendriks, W; Ropers, H H

1996-01-01

In order to elucidate the cellular and molecular processes which are involved in Norrie disease (ND), we have used gene targeting technology to generate ND mutant mice. The murine homologue of the ND gene was cloned and shown to encode a polypeptide that shares 94% of the amino acid sequence with its human counterpart. RNA in situ hybridization revealed expression in retina, brain and the olfactory bulb and epithelium of 2 week old mice. Hemizygous mice carrying a replacement mutation in exon 2 of the ND gene developed retrolental structures in the vitreous body and showed an overall disorganization of the retinal ganglion cell layer. The outer plexiform layer disappears occasionally, resulting in a juxtaposed inner and outer nuclear layer. At the same regions, the outer segments of the photoreceptor cell layer are no longer present. These ocular findings are consistent with observations in ND patients and the generated mouse line provides a faithful model for study of early pathogenic events in this severe X-linked recessive neurological disorder.

Vascular development in the retina and inner ear: control by Norrin and Frizzled-4, a high-affinity ligand-receptor pair.

PubMed

Xu, Qiang; Wang, Yanshu; Dabdoub, Alain; Smallwood, Philip M; Williams, John; Woods, Chad; Kelley, Matthew W; Jiang, Li; Tasman, William; Zhang, Kang; Nathans, Jeremy

2004-03-19

Incomplete retinal vascularization occurs in both Norrie disease and familial exudative vitreoretinopathy (FEVR). Norrin, the protein product of the Norrie disease gene, is a secreted protein of unknown biochemical function. One form of FEVR is caused by defects in Frizzled-4 (Fz4), a presumptive Wnt receptor. We show here that Norrin and Fz4 function as a ligand-receptor pair based on (1) the similarity in vascular phenotypes caused by Norrin and Fz4 mutations in humans and mice, (2) the specificity and high affinity of Norrin-Fz4 binding, (3) the high efficiency with which Norrin induces Fz4- and Lrp-dependent activation of the classical Wnt pathway, and (4) the signaling defects displayed by disease-associated variants of Norrin and Fz4. These data define a Norrin-Fz4 signaling system that plays a central role in vascular development in the eye and ear, and they indicate that ligands unrelated to Wnts can act through Fz receptors.

Twenty years of audiology in a patient with Norrie disease.

PubMed

Halpin, Chris; Sims, Katherine

2008-11-01

To describe disease progression and treatment outcomes over a 20-year period (ages 5-25) in a young man with Norrie disease (occuloacousticocerebral dysplasia), ND; OMIM #310600. Affected individuals are born blind and develop progressive sensory loss with onset in adolescence. This disease is X-linked and has been associated with mutations of the NDP gene (Xp11.4). The patient was followed using repeated audiograms, as well as reports of educational progress and hearing aid use. The specific mutation was found by molecular analysis. The patient demonstrated progressive sensory loss with good preservation of word recognition. The loss was initially high frequency and asymmetric in adolescence and became more severe, more symmetric and affected practically all frequencies by the end of childhood. Educational progress was affected by the cognitive effects of the syndrome, and hearing aid use was very effective. A bilateral progressive sensory loss with good preservation of word recognition was documented in detail. The residual word recognition supported good use of hearing aids in this case.

Cataplexy and monoamine oxidase deficiency in Norrie disease.

PubMed

Vossler, D G; Wyler, A R; Wilkus, R J; Gardner-Walker, G; Vlcek, B W

1996-05-01

Norrie disease (ND) is an X-linked recessive disorder causing ocular atrophy, mental retardation, deafness, and dysmorphic features. Virtually absent monoamine oxidase (MAO) type-A and -B activity has been found in some boys with chromosome deletions. We report the coexistence of cataplexy and abnormal REM sleep organization with ND. Three related boys, referred for treatment of medically refractory atonic spells and apneas, underwent extended EEG-video-polysomnographic monitoring. They demonstrated attacks of cataplexy and inappropriate periods of REM sleep during which they were unarousable. One boy also had generalized tonic-clonic seizures. Previous testing revealed that all three have complete ND gene deletions. In all subjects, platelet MAO-B activity was absent, serum serotonin levels were markedly increased, and plasma catecholamine levels were normal. Data from the canine narcolepsy syndrome model implicate abnormal catecholaminergic and cholinergic activities in the pathogenesis of cataplexy. Our findings suggest that abnormal MAO activity or an imbalance between serotonin and other neurotransmitter levels may be involved in the pathogenesis of human cataplexy.

Genetic factors in human sleep disorders with special reference to Norrie disease, Prader-Willi syndrome and Moebius syndrome.

PubMed

Parkes, J D

1999-06-01

Sleep-wake problems are common in specific inborn errors of metabolism and structure of the central nervous system. Psychological factors, behavioural difficulties, metabolic disturbances, and widespread rather than focal damage to the nervous system are present in many of these diseases and all influence the sleep-wake cycle. However, a number of conditions cause relatively focal damage to the neuroanatomical substrate of sleeping and waking. These include fatal familial insomnia, with involvement of the prion protein gene on chromosome 20, Norrie disease, the Prader-Willi syndrome and the Moebius syndrome. The last three important conditions, although rare, are considered in detail in this review. They result in sensory deprivation, hypothalamic and mid-brain damage, and involve the X-chromosome, chromosome 15, and chromosome 13, respectively. These conditions cause a wide variety of sleep disturbance, including parasomnias, daytime sleepiness, and a condition like cataplexy. The place of the relevant gene products in normal sleep regulation needs further exploration.

Instrument Reporting Practices in Second Language Research

ERIC Educational Resources Information Center

Derrick, Deirdre J.

2016-01-01

Second language (L2) researchers often have to develop or change the instruments they use to measure numerous constructs (Norris & Ortega, 2012). Given the prevalence of researcher-developed and -adapted data collection instruments, and given the profound effect instrumentation can have on results, thorough reporting of instrumentation is…

Books for Educators, Labor Partners, and Children

PubMed Central

Shilling, Teri

2005-01-01

In this column, reviewers offer perspectives and commentary on three books: From Telling to Teaching: A Dialogue Approach to Adult Learning by Joye Norris; The Pocket Doula: A Labor Partner's Guide to Surviving Childbirth by Christine Wallace; and My Mommy's Midwife by Trish Payne and Hayley Holland.

Struggle for Social Position in Digital Media Composition

ERIC Educational Resources Information Center

Doerr-Stevens, Candance

2013-01-01

This study investigates the processes and products of multimodal and multi-authored digital media composition. Using ethnographic case study and Mediated Discourse Analysis (Norris & Jones, 2005), this study focuses specifically on the digital media composition of radio and film documentaries, examining struggle among students, media, and…

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

Thompson, J.M.; Yadav, S.

Waters from geysers, hot springs, and pools of Yellowstone National Park have been analyzed. We report 422 complete major ion analyses from 330 different locations of geysers, hot springs, and pools, collected from 1974 to 1978. Many of the analyses from Upper, Midway, Lower, and Norris Geyser Basin are recollections of features previously reported.

75 FR 2892 - NASA Advisory Council; Science Committee; Heliophysics Subcommittee; Meeting

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2010-01-19

... Science Committee of the NAC. The Meeting will be held for the purpose of soliciting from the scientific... NATIONAL AERONAUTICS AND SPACE ADMINISTRATION [Notice (10-004)] NASA Advisory Council; Science..., Washington, DC 20546. FOR FURTHER INFORMATION CONTACT: Ms. Marian Norris, Science Mission Directorate, NASA...

75 FR 19661 - NASA Advisory Council; Science Committee; Planetary Protection Subcommittee; Meeting

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2010-04-15

...). This Subcommittee reports to the Science Committee of the NAC. The meeting will be held for the purpose... NATIONAL AERONAUTICS AND SPACE ADMINISTRATION [Notice (10-044)] NASA Advisory Council; Science... 20546. FOR FURTHER INFORMATION CONTACT: Ms. Marian Norris, Science Mission Directorate, NASA...

75 FR 30074 - NASA Advisory Council; Science Committee; Heliophysics Subcommittee; Meeting

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2010-05-28

... Science Committee of the NAC. The Meeting will be held for the purpose of soliciting from the scientific... NATIONAL AERONAUTICS AND SPACE ADMINISTRATION [Notice (10-061)] NASA Advisory Council; Science...: Ms. Marian Norris, Science Mission Directorate, NASA Headquarters, Washington, DC 20546, (202) 358...

Enhanced Resources of the SMDC Monitoring Research Program for Source Information and Data Acquisition

DTIC Science & Technology

2007-09-01

Springer locations (red) with satellite imagery illustrating roundoff error in Springer locations (b). Comparison of Rainier Mesa tunnel location of...Burrows et al.; Bolt; IAEA South Pacific Burrows et al.; Bolt; IAEA; AWE India Pokhran Gupta/Pabian; Norris/Arkin North Korea Mount Mantap Richards

The Dynamic Nature of Speech Perception

ERIC Educational Resources Information Center

McQueen, James M.; Norris, Dennis; Cutler, Anne

2006-01-01

The speech perception system must be flexible in responding to the variability in speech sounds caused by differences among speakers and by language change over the lifespan of the listener. Indeed, listeners use lexical knowledge to retune perception of novel speech (Norris, McQueen, & Cutler, 2003). In that study, Dutch listeners made…

Measuring Capacities for Community Resilience

ERIC Educational Resources Information Center

Sherrieb, Kathleen; Norris, Fran H.; Galea, Sandro

2010-01-01

The purpose of this study was to measure the sets of adaptive capacities for Economic Development and Social Capital in the Norris et al. (2008) community resilience model with publicly accessible population indicators. Our approach involved five steps. First, we conducted a literature review on measurements of the capacities. Second, we created…

Hot Spot at Yellowstone

ERIC Educational Resources Information Center

Dress, Abby

2005-01-01

Within this huge national park (over two million acres spread across Wyoming, Montana, and Idaho) are steaming geysers, hot springs, bubbling mudpots, and fumaroles, or steam vents. Drives on the main roads of Yellowstone take tourists through the major hot attractions, which also include Norris Geyser Basin, Upper and Lower Geyser Basin, West…

China and Japan’s Strategic Nuclear Relationship

DTIC Science & Technology

2009-09-01

Nuclear Numerology Chinese Style,” Arms Control Today (March, 2005); Robert S. Norris, “Chinese Nuclear Forces 2008,” Bulletin of the Atomic...Bulletin of the Atomic Scientists, May/June. ———. 2005. “Letters to the Editor: Nuclear Numerology Chinese Style,” Arms Control Today, March

Possible-Word Constraints in Cantonese Speech Segmentation

ERIC Educational Resources Information Center

Yip, Michael C. W.

2004-01-01

A Cantonese syllable-spotting experiment was conducted to examine whether the Possible-Word Constraint (PWC), proposed by Norris, McQueen, Cutler, and Butterfield (1997), can apply in Cantonese speech segmentation. In the experiment, listeners were asked to spot out the target Cantonese syllable from a series of nonsense sound strings. Results…

SAVEWS Jr. User’s Manual, Version 1.0

DTIC Science & Technology

2014-04-01

Botany 53: 227-233. Mueller-Dombois, D., and H. Ellenberg. 1974. Aims and methods of vegetation ecology. New York: John Wiley & Sons, Inc. Norris...videography. Aquatic Botany 58: 269-287. Sabol, B., R. E. Melton, R. Chamberlain, P. Doering, and K. Haunert. 2002. Evaluation of a digital echo

The Role of the Capase-8 Inhibitor FLIP in Androgen-Withdrawal Induced Death of Prostate Epithelium

DTIC Science & Technology

2006-01-01

DL, Norris JS. Resistance of prostate cancer cells to soluble TNF- related apoptosis- inducing ligand (TRAIL/Apo2L) can be overcome by doxorubicin or...Bueso-Ramos C, Chatterjee D, Pantazis P, Aggarwal BB. Curcumin downregulates cell survival mechanisms in human prostate cancer cell lines. Oncogene

The Role of the Caspase-8 Inhibitor FLIP in Androgen-Withdrawal Induced Death of Prostate Epithelium

DTIC Science & Technology

2006-01-01

DL, Norris JS. Resistance of prostate cancer cells to soluble TNF- related apoptosis- inducing ligand (TRAIL/Apo2L) can be overcome by doxorubicin or...Bueso-Ramos C, Chatterjee D, Pantazis P, Aggarwal BB. Curcumin downregulates cell survival mechanisms in human prostate cancer cell lines. Oncogene

Why Teacher Must Go Mobile

ERIC Educational Resources Information Center

Pascopella, Angela

2009-01-01

This article profiles Cathleen Norris, a Regents Professor in the department of Learning Technologies at the University of North Texas and co-creator of GoKnow Learning who encourages teachers to use cell phones in lessons. As a middle and high school math teacher for 14 years in the Norman (Oklahoma) School District and Dallas (Texas) Independent…

On the Influence of Naturalism on American Literature

ERIC Educational Resources Information Center

Zhang, Xiaofen

2010-01-01

Naturalism was first proposed and formulated by French novelist Emile Zola, and it was introduced to America by American novelist Frank Norris. It is a new and harsher realism. It is a theory in literature emphasizing scientific observation of life without idealism or avoidance of the ugly. American literature naturalists dismissed the validity of…

Strategic Studies Quarterly. Volume 5, Number 3. Fall 2011

DTIC Science & Technology

2011-01-01

Christopher M. Hemmer, PhD Kimberly A. Hudson, PhD Col Basil S. Norris Jr., USAF, Retired Gary J. Schaub, PhD Strategic Studies Quarterly (SSQ...29). Sloterdijk goes on to describe a direct progression from the wartime use of poi­ son gas to the postwar development of pesticides and of Zyklon

Aerial Surveys of Bowhead Whales, North Slope, Alaska.

DTIC Science & Technology

1980-02-01

euphausiids. amphipods. copepods, mysids, and pteropods (Lowrey and Burns). While basically "skimmers," they do forage very near the bottom, at least in...1 3o- t 3-7. IDawhin . Wit1. 1960. lire seasonal migrat ion of* huLmpback whles. pp. 14 5- 17(). In: K.S Norris ted.). W hales. dolphirns, aird

76 FR 5405 - NASA Advisory Council; Science Committee; Astrophysics Subcommittee; Meeting

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2011-01-31

..., (202) 358-4452, fax (202) 358-4118, or [email protected]nasa.gov . SUPPLEMENTARY INFORMATION: The meeting will... identifying information 3 working days in advance by contacting Marian Norris via e-mail at [email protected]nasa.gov... NATIONAL AERONAUTICS AND SPACE ADMINISTRATION [Notice (11-012)] NASA Advisory Council; Science...

75 FR 39974 - NASA Advisory Council; Science Committee; Planetary Protection Subcommittee; Meeting

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2010-07-13

..., DC 20546, (202) 358-4452, fax (202) 358-4118, or [email protected]nasa.gov . SUPPLEMENTARY INFORMATION: The... identifying information 3 working days in advance by contacting Marian Norris via e-mail at [email protected]nasa.gov... NATIONAL AERONAUTICS AND SPACE ADMINISTRATION [Notice (10-075)] NASA Advisory Council; Science...

76 FR 21411 - NASA Advisory Council; Science Committee; Planetary Protection Subcommittee; Meeting

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2011-04-15

..., Washington, DC 20546, (202) 358-4452, fax (202) 358-4118, or [email protected]nasa.gov . SUPPLEMENTARY INFORMATION... advance by contacting Marian Norris via e-mail at [email protected]nasa.gov or by telephone at (202) 358-4452... NATIONAL AERONAUTICS AND SPACE ADMINISTRATION [Notice: (11-041)] NASA Advisory Council; Science...

75 FR 80850 - NASA Advisory Council; Science Committee; Planetary Protection Subcommittee; Meeting

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2010-12-23

...) 358-4452, fax (202) 358-4118, or [email protected]nasa.gov . SUPPLEMENTARY INFORMATION: The meeting will be... identifying information 3 working days in advance by contacting Marian Norris via e-mail at [email protected]nasa.gov... NATIONAL AERONAUTICS AND SPACE ADMINISTRATION [Notice: (10-168)] NASA Advisory Council; Science...

Effects of First and Second Language on Segmentation of Non-Native Speech

ERIC Educational Resources Information Center

Hanulikova, Adriana; Mitterer, Holger; McQueen, James M.

2011-01-01

Do Slovak-German bilinguals apply native Slovak phonological and lexical knowledge when segmenting German speech? When Slovaks listen to their native language, segmentation is impaired when fixed-stress cues are absent (Hanulikova, McQueen & Mitterer, 2010), and, following the Possible-Word Constraint (PWC; Norris, McQueen, Cutler & Butterfield,…

76 FR 72197 - Before Commissioners: Jon Wellinghoff, Chairman; Philip D. Moeller, John R. Norris, and Cheryl A...

Federal Register 2010, 2011, 2012, 2013, 2014

2011-11-22

... has not adequately justified its proposed ``lower'' VRF designation for Requirements R1 and R4, and direct NERC to either provide additional justification for these VRF designations or propose a revised VRF designation that addresses our concerns. \\33\\ See North American Electric Reliability Corp., 119...

78 FR 42064 - Before Commissioners: Jon Wellinghoff, Chairman; Philip D. Moeller, John R. Norris, Cheryl A...

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2013-07-15

... reporting is ``an ex post activity'' that provides only prospective benefits to system reliability.\\14... Section 215, and those obligations that do not, such as administrative record-keeping and ex- post... EOP-004-2 becomes effective. Finally, we approve the proposed violation risk factors and violation...

Pseudohomophone Priming in Lexical Decision Is Not Fragile in a Sparse Lexical Neighborhood

ERIC Educational Resources Information Center

Kinoshita, Sachiko; Norris, Dennis

2012-01-01

In lexical decision, to date few studies in English have found a reliable pseudohomophone priming advantage with orthographically similar primes (the "klip-plip effect"; Frost, Ahissar, Gotesman, & Tayeb, 2003; see Rastle & Brysbaert, 2006, for a review). On the basis of the Bayseian reader model of lexical decision (Norris,…

Maternal xNorrin, a Canonical Wnt Signaling Agonist and TGF-β Antagonist, Controls Early Neuroectoderm Specification in Xenopus

PubMed Central

Xu, Suhong; Cheng, Feng; Liang, Juan; Wu, Wei; Zhang, Jian

2012-01-01

Dorsal–ventral specification in the amphibian embryo is controlled by β-catenin, whose activation in all dorsal cells is dependent on maternal Wnt11. However, it remains unknown whether other maternally secreted factors contribute to β-catenin activation in the dorsal ectoderm. Here, we show that maternal Xenopus Norrin (xNorrin) promotes anterior neural tissue formation in ventralized embryos. Conversely, when xNorrin function is inhibited, early canonical Wnt signaling in the dorsal ectoderm and the early expression of the zygotic neural inducers Chordin, Noggin, and Xnr3 are severely suppressed, causing the loss of anterior structures. In addition, xNorrin potently inhibits BMP- and Nodal/Activin-related functions through direct binding to the ligands. Moreover, a subset of Norrin mutants identified in humans with Norrie disease retain Wnt activation but show defective inhibition of Nodal/Activin-related signaling in mesoderm induction, suggesting that this disinhibition causes Norrie disease. Thus, xNorrin is an unusual molecule that acts on two major signaling pathways, Wnt and TGF-β, in opposite ways and is essential for early neuroectoderm specification. PMID:22448144

Correlation of ophthalmic examination with carrier status in females potentially harboring a severe Norrie disease gene mutation.

PubMed

Khan, Arif O; Aldahmesh, Mohammed A; Meyer, Brian

2008-04-01

To correlate ophthalmic findings with carrier status for a severe Norrie disease (ND) gene mutation (C95F). Prospective interventional case series. Six potential carriers and 1 obligate carrier from a family harboring the mutation. An ophthalmologist blind to the pedigree performed a full ophthalmic examination for the 7 asymptomatic family members. A peripheral blood sample was collected from each for ND gene sequencing. Ophthalmic examination findings (with attention to the presence or absence of retinal findings) and results of ND gene sequencing. Three carriers were identified by molecular genetics, and all 3 of them had peripheral retinal abnormality. However, 3 of the 4 genetically identified noncarriers also exhibited peripheral retinal abnormality. Two of these noncarriers with retinal findings were the offspring of a confirmed noncarrier. The genetically identified noncarrier with a normal peripheral retinal examination was the daughter of an obligate carrier. The presence of peripheral retinal changes was not useful for carrier prediction in a family harboring ND. There are likely additional loci responsible for phenotypic expression.

Next-generation sequencing reveals a novel NDP gene mutation in a Chinese family with Norrie disease.

PubMed

Huang, Xiaoyan; Tian, Mao; Li, Jiankang; Cui, Ling; Li, Min; Zhang, Jianguo

2017-11-01

Norrie disease (ND) is a rare X-linked genetic disorder, the main symptoms of which are congenital blindness and white pupils. It has been reported that ND is caused by mutations in the NDP gene. Although many mutations in NDP have been reported, the genetic cause for many patients remains unknown. In this study, the aim is to investigate the genetic defect in a five-generation family with typical symptoms of ND. To identify the causative gene, next-generation sequencing based target capture sequencing was performed. Segregation analysis of the candidate variant was performed in additional family members using Sanger sequencing. We identified a novel missense variant (c.314C>A) located within the NDP gene. The mutation cosegregated within all affected individuals in the family and was not found in unaffected members. By happenstance, in this family, we also detected a known pathogenic variant of retinitis pigmentosa in a healthy individual. c.314C>A mutation of NDP gene is a novel mutation and broadens the genetic spectrum of ND.

Identification of missense mutations in the Norrie disease gene associated with advanced retinopathy of prematurity.

PubMed

Shastry, B S; Pendergast, S D; Hartzer, M K; Liu, X; Trese, M T

1997-05-01

Retinopathy of prematurity (ROP) is a retinal vascular disease occurring in infants with short gestational age and low birth weight and can lead to retinal detachment (ROP stages 4 and 5). X-linked familial exudative vitreoretinopathy is phenotypically similar to ROP and has been associated with mutations in the Norrie disease (ND) gene in some cases. To determine if similar mutations in the ND gene may play a role in the development of advanced ROP. Clinical examination and molecular genetic analysis were performed on 16 children, including 2 dizygotic and 1 monozygotic twin pairs, and their parents from 13 families. Sequencing of the amplified products revealed missense mutations (R121W and L108P) in the third exon of the ND gene in 4 patients. These mutations were not present in an unaffected premature twin, 2 children with regressed stage 3 ROP, the parents, or in 50 unrelated healthy control subjects. These findings suggest that mutations in the ND gene may play a role in the development of severe ROP in premature infants.

Fatty acid composition from the marine red algae Pterocladiella capillacea (S. G. Gmelin) Santelices & Hommersand 1997 and Osmundaria obtusiloba (C. Agardh) R. E. Norris 1991 and its antioxidant activity.

PubMed

Alencar, Daniel B DE; Diniz, Jaécio C; Rocha, Simone A S; Pires-Cavalcante, Kelma M S; Lima, Rebeca L DE; Sousa, Karolina C DE; Freitas, Jefferson O; Bezerra, Rayssa M; Baracho, Bárbara M; Sampaio, Alexandre H; Viana, Francisco A; Saker-Sampaio, Silvana

2018-01-01

This study evaluated the chemical composition and antioxidant activity of fatty acids from the marine red algae Pterocladiella capillacea (S. G. Gmelin) Santelices & Hommersand 1997 and Osmundaria obtusiloba (C. Agardh) R. E. Norris 1991. The gas chromatography mass spectrometry (GC-MS) identified nine fatty acids in the two species. The major fatty acids of P. capillacea and O. obtusiloba were palmitic acid, oleic acid, arachidonic acid and eicosapentaenoic acid. The DPPH radical scavenging capacity of fatty acids was moderate ranging from 25.90% to 29.97%. Fatty acids from P. capillacea (31.18%) had a moderate ferrous ions chelating activity (FIC), while in O. obtusiloba (17.17%), was weak. The ferric reducing antioxidant power (FRAP) of fatty acids from P. capillacea and O. obtusiloba was low. As for β-carotene bleaching (BCB), P. capillacea and O. obtusiloba showed a good activity. This is the first report of the antioxidant activities of fatty acids from the marine red algae P. capillacea and O. obtusiloba.

A novel NDP mutation in an infant with unilateral persistent fetal vasculature and retinal vasculopathy.

PubMed

Aponte, Elisabeth P; Pulido, Jose S; Ellison, Jay W; Quiram, Polly A; Mohney, Brian G

2009-06-01

Mutations in the Norrie Disease gene, Norrie Disease Pseudoglioma (NDP) lead to a phenotypically heterogeneous group of retinopathies. We report a novel mutation in the NDP gene identified in a patient whose clinical presentation was suggestive of unilateral persistent fetal vasculature (PFV). Ophthalmic examinations, ocular ultrasounds and sequence analysis of the exons of the NDP gene on peripheral blood DNA were performed. A four-month-old boy was referred to our institution for presumed unilateral retinoblastoma. The clinical and ultrasonographic exams were consistent with PFV and retinal detachment of the left eye as well as retinal fibrovascular changes in the right eye. A vitrectomy of the left eye revealed the absence of a retrolenticular stalk and mutation analysis of the NDP gene of the proband and mother demonstrated a novel missense mutation at codon 66, designated as c. 196G > A at the cDNA level and E66K at the protein level. We report a novel mutation in the NDP gene in a patient whose presentation demonstrates the phenotypic heterogeneity of NDP-related disorders.

Next-generation sequencing reveals a novel NDP gene mutation in a Chinese family with Norrie disease

PubMed Central

Huang, Xiaoyan; Tian, Mao; Li, Jiankang; Cui, Ling; Li, Min; Zhang, Jianguo

2017-01-01

Purpose: Norrie disease (ND) is a rare X-linked genetic disorder, the main symptoms of which are congenital blindness and white pupils. It has been reported that ND is caused by mutations in the NDP gene. Although many mutations in NDP have been reported, the genetic cause for many patients remains unknown. In this study, the aim is to investigate the genetic defect in a five-generation family with typical symptoms of ND. Methods: To identify the causative gene, next-generation sequencing based target capture sequencing was performed. Segregation analysis of the candidate variant was performed in additional family members using Sanger sequencing. Results: We identified a novel missense variant (c.314C>A) located within the NDP gene. The mutation cosegregated within all affected individuals in the family and was not found in unaffected members. By happenstance, in this family, we also detected a known pathogenic variant of retinitis pigmentosa in a healthy individual. Conclusion: c.314C>A mutation of NDP gene is a novel mutation and broadens the genetic spectrum of ND. PMID:29133643

Disaster averted: Community resilience in the face of a catastrophic flood.

PubMed

O'Neill, H Katherine; McLean, Andrew J; Kalis, Renetta; Shultz, James M

2016-01-01

In the spring of 2009, the Fargo, North Dakota, metropolitan area had 5 days to lay millions of sandbags to avoid devastation from record flooding of the Red River of the North. The community was able to successfully mitigate the flooding and escape potentially catastrophic economic, physical, and mental health consequences. We hypothesized that Fargo flood protection efforts reflected the community resilience factors proposed by Norris, Stevens, Pfefferbaum, et al. (2008): citizen involvement in mitigation efforts, effective organizational linkages, ongoing psychosocial support, and strong civic leadership in the face of rapidly changing circumstances. This community case report utilizes an extensive review of available sources, including news reports, government documents, research articles, and personal communication. Results demonstrate that Fargo's response to the threat of catastrophic flooding was consistent with Norris et al.'s (2008) factors of community resilience. Furthermore, success in 2009 carried over into future flood prevention and response efforts, as well as a structured approach to building psychological resilience. This case study contributes to the literature on community resilience by describing a community's successful efforts to avert a potentially catastrophic disaster.

Disaster averted: Community resilience in the face of a catastrophic flood

PubMed Central

O'Neill, H. Katherine; McLean, Andrew J.; Kalis, Renetta; Shultz, James M.

2016-01-01

ABSTRACT In the spring of 2009, the Fargo, North Dakota, metropolitan area had 5 days to lay millions of sandbags to avoid devastation from record flooding of the Red River of the North. The community was able to successfully mitigate the flooding and escape potentially catastrophic economic, physical, and mental health consequences. We hypothesized that Fargo flood protection efforts reflected the community resilience factors proposed by Norris, Stevens, Pfefferbaum, et al. (2008): citizen involvement in mitigation efforts, effective organizational linkages, ongoing psychosocial support, and strong civic leadership in the face of rapidly changing circumstances. This community case report utilizes an extensive review of available sources, including news reports, government documents, research articles, and personal communication. Results demonstrate that Fargo's response to the threat of catastrophic flooding was consistent with Norris et al.'s (2008) factors of community resilience. Furthermore, success in 2009 carried over into future flood prevention and response efforts, as well as a structured approach to building psychological resilience. This case study contributes to the literature on community resilience by describing a community's successful efforts to avert a potentially catastrophic disaster. PMID:28229016

Contiguous deletion of the NDP, MAOA, MAOB, and EFHC2 genes in a patient with Norrie disease, severe psychomotor retardation and myoclonic epilepsy.

PubMed

Rodriguez-Revenga, L; Madrigal, I; Alkhalidi, L S; Armengol, L; González, E; Badenas, C; Estivill, X; Milà, M

2007-05-01

Norrie disease (ND) is an X-linked disorder, inherited as a recessive trait that, therefore, mostly affects males. The gene responsible for ND, called NDP, maps to the short arm of chromosome X (Xp11.4-p11.3). We report here an atypical case of ND, consisting of a patient harboring a large submicroscopic deletion affecting not only the NDP gene but also the MAOA, MAOB, and EFHC2 genes. Microarray comparative genomic hybridization (CGH) analysis showed that 11 consecutive bacterial artificial chromosome (BAC) clones, mapping around the NDP gene, were deleted. These clones span a region of about 1 Mb on Xp11.3. The deletion was ascertained by fluorescent in situ hybridization (FISH) analysis with different BAC clones located within the region. Clinical features of the proband include bilateral retinal detachment, microcephaly, severe psychomotor retardation without verbal language skills acquired, and epilepsy. The identification and molecular characterization of this case reinforces the idea of a new contiguous gene syndrome that would explain the complex phenotype shared by atypical ND patients.

Securities and Exchange Commission Semiannual Regulatory Agenda

Federal Register 2010, 2011, 2012, 2013, 2014

2010-12-20

... USC 78n(d)(4); 15 USC 78o(c)(3); 15 USC 78o(c)(6); 15 USC 78q-1(a); 15 USC 78q- 1(d)(1); 15 USC 78w(a...: 15 USC 78b; 15 USC 78c(b); 15 USC 78j; 15 USC 78o(c); 15 USC 78o-4; 15 USC 78q; 15 USC 78w(a)(1... official business days between the hours of 10:00 a.m. and 3:00 p.m. All comments received will be posted...

20. MEMBER 'A' SHOWS TENON AS USED IN POST 'A' ...

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

20. MEMBER 'A' SHOWS TENON AS USED IN POST 'A' (TN-159A-19), MEMBER 'B' IS BEAM 'B' IN TN-159A-19 AND SHOWS METHOD OF JOINING THESE MEMBERS. MEMBER 'C' SHOWS MORTISE IN BEAM 'B'. - Caleb Crosby Threshing Barn, Noeton (moved to Norris Dam State Park, Lake City), Morristown, Hamblen County, TN

MX, A DRINKING WATER CARCINOGEN, DOES NOT INDUCE MUTATIONS IN THE LIVER OF CII TRANSGENIC MEDAKA

EPA Science Inventory

MX, a drinking water carcinogen, does not induce mutations in the liver of cII transgenic medaka
Geter, DR; Winn, RN; Fournie, JW; Norris, MB; DeAngelo, AB; and Hawkins, WE

Abstract

Geter et al., page 2
Abstract
"Salmonella" mutagenicity assays have shown t...

Russell, Henry Norris (1877-1957)

NASA Astrophysics Data System (ADS)

Murdin, P.

2000-11-01

Astronomer, born in Oyster Bay, NY, spent nearly all his life working at Princeton University. He spectroscopically studied eclipsing binary stars to determine the masses of their component stars. At first collaborating with the British astronomer Hinks at Cambridge, he started to measure stellar parallaxes and, plotting the absolute magnitudes of stars whose distance he had thus measured, agains...

75 FR 44893 - Pipeline Posting Requirements Under Section 23 of the Natural Gas Act

Federal Register 2010, 2011, 2012, 2013, 2014

2010-07-30

... and RM08-2-000; Order No. 720-B] Pipeline Posting Requirements Under Section 23 of the Natural Gas Act... R. Norris, and Cheryl A. LaFleur. Pipeline Posting Requirements under Section 23 of the Natural Gas...) issued Order No. 720 requiring interstate and certain major non-interstate natural gas pipelines to post...

A Game of Simon Says: Latin America’s Left Turn and Its Effects on US Security

DTIC Science & Technology

2008-05-01

Fred P. Stone, Lt Col, PhD, Director of Research John T. Ackerman, PhD, Series Editor Kathleen Mahoney-Norris, PhD, Essay Advisor Air University...lopsided than even the lyrics from the 966 Beatles’ song “Taxman” envisioned. Alvaro Hurtado of the Interna- tional Labour Organization states that

Quality of Functional Return in Limbs and Tissues Replanted and Transplanted by Microsurgical Techniques.

DTIC Science & Technology

1981-06-15

app.lications and functional esults of microneurov ascu icr transplants of neurovascua island flaps, muscles,di gits and toes are included D0 or 0147...Plastic Surgery, 33:383, 1980. 54. Microvascular Procedure in Patients Over Fifty Years of Age ; G.D. Harris, F.Finseth, H.J. Buncke, T.R. Norris:Chirurgia

Roll Splitting of Biomass

Treesearch

Colin Ashmore

1984-01-01

This presentation is to make you aware of a biomass harvesting research project just initiated between TVA in Norris, Tennessee and the U.S. Forest Service in Auburn, Alabama, and hopefully, to generate your and your company's input in the form of questions, comments, and concerns, to problems you may have experienced in your rights-of-way (ROW) management program...

Dynamic Development of Complexity and Accuracy: A Case Study in Second Language Academic Writing

ERIC Educational Resources Information Center

Rosmawati

2014-01-01

This paper reports on the development of complexity and accuracy in English as a Second Language (ESL) academic writing. Although research into complexity and accuracy development in second language (L2) writing has been well established, few studies have assumed the multidimensionality of these two constructs (Norris & Ortega, 2009) or…

Sight-Reading Requirements at Concert Band Festivals: A National Survey

ERIC Educational Resources Information Center

Paul, Timothy A.

2010-01-01

This study, a replication and extension of work by Norris (2004), examined sight-reading requirements at middle and high school large-group band festivals across the United States. As in the earlier investigation, answers to the following questions were solicited from all 50 states: (1) Are there ratings-based large-group band festivals? (2) Is…

Text of the U.S. Court of Appeals Opinion in the Spirt Equal-Pension Case.

ERIC Educational Resources Information Center

Newman, Jon O.; And Others

1984-01-01

The opinion of a three-judge panel in a court case involving the validity of gender-based mortality tables and the right of women to receive equal pensions from the Teachers Insurance Annuities Association and the College Retirement Equities Fund is presented, including references to the earlier, related Norris case. (MSE)

76 FR 69768 - NASA Advisory Council; Science Committee Planetary Protection Subcommittee; Meeting

Federal Register 2010, 2011, 2012, 2013, 2014

2011-11-09

...) 358-4118, or [email protected]nasa.gov . SUPPLEMENTARY INFORMATION: The meeting will be open to the public up... November 18, 2011, to Marian Norris via email at [email protected]nasa.gov or by telephone at (202) 358-4452... NATIONAL AERONAUTICS AND SPACE ADMINISTRATION [Notice 11-114] NASA Advisory Council; Science...

78 FR 36276 - NASA Advisory Council; Science Committee; Astrophysics Subcommittee; Meeting

Federal Register 2010, 2011, 2012, 2013, 2014

2013-06-17

..., NASA Headquarters, Washington, DC 20546, (202) 358-4452 or [email protected]nasa.gov . SUPPLEMENTARY INFORMATION... Marian Norris via email at [email protected]nasa.gov no later than close of business July 1, 2013. If the above... or email [email protected]nasa.gov . Patricia D. Rausch, Advisory Committee Management Officer, National...

Assessing Second-Language Oral Proficiency for Research: The Spanish Elicited Imitation Task

ERIC Educational Resources Information Center

Bowden, Harriet Wood

2016-01-01

Proficiency is a key variable in late second language (L2) learning, but one that is undermeasured in current research. This study investigates whether L2 oral proficiency can be quickly and reliably assessed via the Spanish "elicited imitation task" (EIT; Ortega, Iwashita, Rabie, & Norris, 1999). Thirty-seven L2 learners of Spanish…

Minister's Forum on Learning: Learning for Life. What We Heard (Edmonton, Alberta, Canada, November 19-20, 1999).

ERIC Educational Resources Information Center

Alberta Learning, Edmonton.

This report provides a chronological overview of activities of the 1999 Minister's Forum. It summarizes the keynote speech, "Transforming Learning in the Knowledge Age" (Donald Norris), the discussion that occurred among the panelists, and the participants' responses to three topics during small group discussions. The report recaps topic…

What Should Be Explicit in Explicit Grammar Instruction?

ERIC Educational Resources Information Center

Nagai, Noriko; Ayano, Seiki; Okada, Keiko; Nakanishi, Takayuki

2015-01-01

This article proposes an approach to explicit grammar instruction that seeks to develop metalinguistic knowledge of the L2 and raise L2 learners' awareness of their L1, which is crucial for the success of second language acquisition (Ellis 1997, 2002). If explicit instruction is more effective than implicit instruction (Norris and Ortega 2000),…

4/16: Public Tragedy Collides with Personal Trauma

ERIC Educational Resources Information Center

Tilley-Lubbs, Gresilda A.

2011-01-01

When the massacre of 32 students plus the suicide of the shooter occurred at Virginia Tech on April 16, 2007, this author watched the external events from the relative safety of her office located directly across the Drillfield from Norris Hall, where the shootings occurred. Subsequently, the author fell into a deep state of depression following…

76 FR 22448 - Delays in Processing of Special Permits Applications

Federal Register 2010, 2011, 2012, 2013, 2014

2011-04-21

..., AR. 8826-M Phoenix Air Group, Cartersville, 4 05-31-2011 GA. 10869-M Norris Cylinder Company, 4 05-31.... 10656-M Conference of Radiation Control 4 06-30-2011 Program Directors, Inc., Frankfort, KY. 11406-M Conference of Radiation Control 4 06-30-2011 Program Directors, Inc., Frankfort, KY. 14854-M Airgas, Inc...

Genotype-Phenotype Characterization of Novel Variants in Six Italian Patients with Familial Exudative Vitreoretinopathy.

PubMed

Iarossi, Giancarlo; Bertelli, Matteo; Maltese, Paolo Enrico; Gusson, Elena; Marchini, Giorgio; Bruson, Alice; Benedetti, Sabrina; Volpetti, Sabrina; Catena, Gino; Buzzonetti, Luca; Ziccardi, Lucia

2017-01-01

Familial exudative vitreoretinopathy (FEVR) is a complex disorder characterized by incomplete development of the retinal vasculature. Here, we report the results obtained on the spectrum of genetic variations and correlated phenotypes found in a cohort of Italian FEVR patients. Eight probands (age range 7-19 years) were assessed by genetic analysis and comprehensive age-appropriate ophthalmic examination. Genetic testing investigated the genes most widely associated in literature with FEVR: FZD4 , LRP5 , TSPAN12 , and NDP . Clinical and genetic evaluations were extended to relatives of probands positive to genetic testing. Six out of eight probands (75%) showed a genetic variation probably related to the phenotype. We identified four novel genetic variants, one variant already described in association with Norrie disease and one previously described linked to autosomal dominant FEVR. Pedigree analysis of patients led to the classification of four autosomal dominant cases of FEVR (caused by FZD4 and TSPAN12 variants) and two X-linked FEVR probands ( NDP variants). None of the patients showed variants in the LRP5 gene. This study represents the largest cohort study in Italian FEVR patients. Our findings are in agreement with the previous literature confirming that FEVR is a clinically and genetically heterogeneous retinal disorder, even when it manifests in the same family.

Genotype-Phenotype Characterization of Novel Variants in Six Italian Patients with Familial Exudative Vitreoretinopathy

PubMed Central

Marchini, Giorgio; Volpetti, Sabrina; Catena, Gino

2017-01-01

Familial exudative vitreoretinopathy (FEVR) is a complex disorder characterized by incomplete development of the retinal vasculature. Here, we report the results obtained on the spectrum of genetic variations and correlated phenotypes found in a cohort of Italian FEVR patients. Eight probands (age range 7–19 years) were assessed by genetic analysis and comprehensive age-appropriate ophthalmic examination. Genetic testing investigated the genes most widely associated in literature with FEVR: FZD4, LRP5, TSPAN12, and NDP. Clinical and genetic evaluations were extended to relatives of probands positive to genetic testing. Six out of eight probands (75%) showed a genetic variation probably related to the phenotype. We identified four novel genetic variants, one variant already described in association with Norrie disease and one previously described linked to autosomal dominant FEVR. Pedigree analysis of patients led to the classification of four autosomal dominant cases of FEVR (caused by FZD4 and TSPAN12 variants) and two X-linked FEVR probands (NDP variants). None of the patients showed variants in the LRP5 gene. This study represents the largest cohort study in Italian FEVR patients. Our findings are in agreement with the previous literature confirming that FEVR is a clinically and genetically heterogeneous retinal disorder, even when it manifests in the same family. PMID:28758032

U. S. statutes of general interest to safeguards and security officers

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

Cadwell, J.J.

1988-01-01

A handbook of enforcement provisions of Federal law and regulations was prepared for use by U.S. DOE Security Inspectors. This handbook provides security inspectors for the U.S. Department of Energy, security officers at Nuclear Regulatory Licensee facilities, and others with a single document containing most of the Federal law provisions available to assist them in enforcing agency regulations. The handbook contains selected enforcement provisions of Titles 18, 42 and 50 of the United States Code (USC). Topical coverage of Title 18 includes Espionage and Misrepresentation or Impersonation; Theft and Embezzlement; Malicious Mischief; Conspiracy; Search and Seizure. A miscellaneous section dealsmore » with explosives, blackmail, firearms, and other subjects. Certain enforcement sections of Title 42 of the USC (The Atomic Energy Act) and of the Internal Security Act of the United States Code (Title 50) are also provided. Finally, relevant parts of the Federal Property Management Regulations of Title 50, Chapter 101 of the Code of Federal Regulations are presented. A comprehensive index is provided based on key words.« less

Papers and Proceedings. Syntopican VIII: "Moving Information--Concepts in Transition." (Minneapolis, MN, June 23-26, 1980).

ERIC Educational Resources Information Center

1980

This collection of 22 papers examines various word processing (WP) technologies, systems, and applications. The first five papers by C. Briggs, C. Taylor, G. McLean, D. Remsen, and C. Norris discuss WP applications in the Army, a WP system for an insurance firm, the organization of the International Word Processing Association, WP fundamentals,…

Biodiversity and Peace: Where Technology and Montessori Come Together in the Children's Eternal Rainforest, Costa Rica

ERIC Educational Resources Information Center

Norris, Jeff

2016-01-01

Jeff Norris, initially shocked by the Montessorians who are calling technology into question, states that technology can offer a means of development for the child who is concurrently supporting and learning from the rich and overpowering biodiversity of the rainforest. He speaks for the Children's Eternal Rainforest citizen's science as well as…

THE "MUD VOLCANO," A STINKY THERMAL FEATURE ON THE GRAND ...

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

THE "MUD VOLCANO," A STINKY THERMAL FEATURE ON THE GRAND LOOP ROAD. ACIDIC HOT SPRINGS HAVE REDUCED THE UNDERLYING LAVA TO A FINE CLAY, PRODUCING AN AREA OF BOILING MUD. THE ODOR OF ROTTEN EGGS IS FROM HYDROGEN SULFIDE GAS. - Grand Loop Road, Forming circuit between Mammoth Hot Springs, Norris Junction, Madison Junction, Old Faithful, Mammoth, Park County, WY

The Effectiveness of Structured Input and Structured Output on the Acquisition of Japanese Comparative Sentences

ERIC Educational Resources Information Center

Yamashita, Taichi; Iizuka, Takehiro

2017-01-01

Discussion of the roles of input and output has been attracting a number of researchers in second language acquisition (e.g., DeKeyser, 2007; Doughty, 1991; Krashen, 1982; Long, 1983; Norris & Ortega, 2000; Swain, 2000), and VanPatten (2004) advocated that both structured input and structured output allow learners to process input properly.…

To Resign or Not Resign...The Use of Senior Officer Retirements as a Political Tool

DTIC Science & Technology

2012-02-01

2009), 141-161. Mahoney -Norris, Kathleen A. “Generals and Security Policy Issues: Acquiesce, Resign, or Retire?” Paper presented for the American... Newman , Richard J. “A General Salutes by Quitting,” U.S. News and World Report. Vol. 123, Issue 6, p 5. Owens, Mackubin. “The War on Terror and

REPORT ON ACTIVITY OF TASK FORCE 1 IN THE LIFE CYCLE INVENTORY PROGRAMME: DATA REGISTRY - GLOBAL LIFE CYCLE INVENTORY DATA RESOURCES

EPA Science Inventory

This paper presents a summary of the findings of a report prepared by Task Force 1 of the UNEP/SETAC Life Cycle Initiative on the available Life Cycle Inventory (LCI) databases around the world. An update of a previous summary prepared in May 2002 by Norris and Notten, the repor...

Syntactic Complexity Measures and Their Relation to Oral Proficiency in Japanese as a Foreign Language

ERIC Educational Resources Information Center

Iwashita, Noriko

2006-01-01

The study reported in this article is a part of a large-scale study investigating syntactic complexity in second language (L2) oral data in commonly taught foreign languages (English, German, Japanese, and Spanish; Ortega, Iwashita, Rabie, & Norris, in preparation). In this article, preliminary findings of the analysis of the Japanese data are…

Pedagogical Effectiveness and Feasibility of Focus on Form vs. Focus on Meaning in a Reading Class: Compatibility of Teacher-Learner's Perspectives

ERIC Educational Resources Information Center

Abbasian, Gholam-Reza; Pooshaneh, Leila

2015-01-01

Many empirical investigations have demonstrated that explicit Focus on Form (FOF) methods are more effective than implicit Focus on Meaning (FOM) methods (Norris & Ortega, 2000), because in FOF instruction learners' attention is drawn to linguistic form while FOM instruction requires learners' attention to communicate (Ellis, 2001). However,…

75 FR 79873 - Semiannual Regulatory Agenda

Federal Register 2010, 2011, 2012, 2013, 2014

2010-12-20

... Legal Authority: 40 USC 121(c); 10 USC ch 137; 42 USC 2473(c) Abstract: The Civilian Agency Acquisition... REPORTING PROCEDURES Legal Authority: 40 USC 121(c); 10 USC ch 137; 42 USC 2473(c) Abstract: The Civilian... EXECUTIVE COMPENSATION AND FIRST-TIER SUBCONTRACT AWARDS Legal Authority: 40 USC 121(c); 10 USC ch 137; 42...

Linkage and candidate gene analysis of X-linked familial exudative vitreoretinopathy.

PubMed

Shastry, B S; Hejtmancik, J F; Plager, D A; Hartzer, M K; Trese, M T

1995-05-20

Familial exudative vitreoretinopathy (FEVR) is a hereditary eye disorder characterized by avascularity of the peripheral retina, retinal exudates, tractional detachment, and retinal folds. The disorder is most commonly transmitted as an autosomal dominant trait, but X-linked transmission also occurs. To initiate the process of identifying the gene responsible for the X-linked disorder, linkage analysis has been performed with three previously unreported three- or four-generation families. Two-point analysis showed linkage to MAOA (Zmax = 2.1, theta max = 0) and DXS228 (Zmax = 0.5, theta max = 0.11), and this was further confirmed by multipoint analysis with these same markers (Zmax = 2.81 at MAOA), which both lie near the gene causing Norrie disease. Molecular genetic analysis further reveals a missense mutation (R121W) in the third exon of the Norrie's disease gene that perfectly cosegregates with the disease through three generations in one family. This mutation was not detected in the unaffected family members and six normal unrelated controls, suggesting that it is likely to be the pathogenic mutation. Additionally, a polymorphic missense mutation (H127R) was detected in a severely affected patient.

Retinal vascular abnormalities and dragged maculae in a carrier with a new NDP mutation (c.268delC) that caused severe Norrie disease in the proband.

PubMed

Lin, Phoebe; Shankar, Suma P; Duncan, Jacque; Slavotinek, Anne; Stone, Edwin M; Rutar, Tina

2010-02-01

Norrie disease (ND) is caused by mutations in the ND pseudoglioma (NDP) gene (MIM 300658) located at chromosome Xp11.4-p11.3. ND is characterized by abnormal retinal vascular development and vitreoretinal disorganization presenting at birth. Systemic manifestations include sensorineural deafness, progressive mental disorder, behavioral and psychological problems, growth failure, and seizures. Other vitreoretinopathies that are associated with NDP gene mutations include X-linked familial exudative vitreoretinopathy, Coats disease, persistent fetal vasculature, and retinopathy of prematurity. Phenotypic variability associated with NDP gene mutations has been well documented in affected male patients. However, there are limited data on signs in female carriers, with mild peripheral retinal abnormalities reported in both carrier and noncarrier females of families with NDP gene mutations. Here, we report a family harboring a single base-pair deletion, c.268delC, in the NDP gene causing a severe ND phenotype in the male proband and peripheral retinal vascular abnormalities with dragged maculae similar to those observed in familial exudative vitreoretinopathy in his carrier mother. Copyright (c) 2010 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.

Multi-functional norrin is a ligand for the LGR4 receptor

PubMed Central

Deng, Cheng; Reddy, Pradeep; Cheng, Yuan; Luo, Ching-Wei; Hsiao, Chih-Lun; Hsueh, Aaron J. W.

2013-01-01

Summary Mammalian LGR4, 5 and 6 are seven-transmembrane receptors that are important for diverse physiological processes. These receptors are orthologous to DLGR2, a Drosophila receptor activated by the burs/pburs heterodimer important for morphogenesis. Although recent studies indicated that four R-spondin proteins are cognate ligands for LGR4, 5 and 6 receptors, several BMP antagonists in vertebrates have been postulated to be orthologous to burs and pburs. Using newly available genome sequences, we showed that norrin is a vertebrate ortholog for insect burs and pburs and stimulates Wnt signaling mediated by LGR4, but not by LGR5 and 6, in mammalian cells. Although norrin could only activate LGR4, binding studies suggested interactions between norrin and LGR4, 5 and 6. Norrin, the Norrie disease gene product, is also capable of activating Wnt signaling mediated by the Frizzled4 receptor and serves as a BMP antagonist. Mutagenesis studies indicated that different norrin mutations found in patients with Norrie disease can be categorized into subgroups according to defects for signaling through the three distinct binding proteins. Thus, norrin is a rare ligand capable of binding three receptors/binding proteins that are important for BMP and Wnt signaling pathways. PMID:23444378

Multi-functional norrin is a ligand for the LGR4 receptor.

PubMed

Deng, Cheng; Reddy, Pradeep; Cheng, Yuan; Luo, Ching-Wei; Hsiao, Chih-Lun; Hsueh, Aaron J W

2013-05-01

Mammalian LGR4, 5 and 6 are seven-transmembrane receptors that are important for diverse physiological processes. These receptors are orthologous to DLGR2, a Drosophila receptor activated by the burs/pburs heterodimer important for morphogenesis. Although recent studies indicated that four R-spondin proteins are cognate ligands for LGR4, 5 and 6 receptors, several BMP antagonists in vertebrates have been postulated to be orthologous to burs and pburs. Using newly available genome sequences, we showed that norrin is a vertebrate ortholog for insect burs and pburs and stimulates Wnt signaling mediated by LGR4, but not by LGR5 and 6, in mammalian cells. Although norrin could only activate LGR4, binding studies suggested interactions between norrin and LGR4, 5 and 6. Norrin, the Norrie disease gene product, is also capable of activating Wnt signaling mediated by the Frizzled4 receptor and serves as a BMP antagonist. Mutagenesis studies indicated that different norrin mutations found in patients with Norrie disease can be categorized into subgroups according to defects for signaling through the three distinct binding proteins. Thus, norrin is a rare ligand capable of binding three receptors/binding proteins that are important for BMP and Wnt signaling pathways.

Is there still a TRACE of trace?

NASA Astrophysics Data System (ADS)

McClelland, James; Mirman, Daniel; Holt, Lori

2003-04-01

According to the TRACE model [McClelland and Elman, Cogn. Psychol. 18, 1-86 (1986)], speech recognition is an interactive activation process involving the integrated use of top-down (lexical) and bottom-up (acoustic) information. Although it is widely accepted that there are lexical influences on speech perception, there has been a disagreement over their exact nature. Two contested predictions of TRACE are that (a) lexical influences should delay or inhibit recognition of phonemes not consistent with lexical information and (b) a lexical influence on the identification of one phoneme can trigger compensation for co-articulation, affecting the identification of other phonemes. Others [Norris, McQueen, and Cutler, BBS 23, 299-370 (2000)] have argued that the predicted effects do not occur, taking this to support an alternative to the TRACE model in which lexical influences do not affect perception, but only a post-perceptual identification process. We re-examine the evidence on these points along with the recent finding that lexical information may lead to a lasting adjustment of category boundaries [McQueen, Norris, and Cutler, Psychonomics Abstract 255 (2001)]. Our analysis indicates that the existing evidence is completely consistent with TRACE, and we suggest additional research that will be necessary to resolve unanswered questions.

Trends in the Types of Usual Sources of Care: A Shift from People to Places or Nothing at All.

PubMed

Liaw, Winston; Jetty, Anuradha; Petterson, Stephen; Bazemore, Andrew; Green, Larry

2017-08-31

(1) To examine usual source of care (USC) trends across four categories (No USC, Person USC, Person, in Facility USC, and Facility USC), and (2) to determine whether USC types are associated with emergency department (ED) visits and hospital admissions. 1996-2014 Medical Expenditure Panel Surveys. We stratified each USC category, by age, region, gender, poverty, insurance, race/ethnicity, and education and used regression to determine the characteristics associated with USC types, ED visits, and hospital admissions. Those with No USC and Facility USCs increased 10 and 18 percent, respectively, while those with Person USCs decreased by 43 percent. Compared to those in the lowest income bracket, those in the highest income bracket were less likely to have a Facility USC. Among those with low incomes, individuals with No USC, Person, in Facility, and Facility USCs were more likely to have ED visits than those with Person USCs. A growing number are reporting facilities as their USCs or none at all. The impact of these trends is uncertain, although we found that some USC types are associated with ED visits and hospital admissions. Tracking USCs will be crucial to measuring progress toward enhanced care efficiency. © Health Research and Educational Trust.

Early-middle Eocene transition in calcareous nannofossil assemblages at IODP Site U1410 (Southeast Newfoundland Ridge, NW Atlantic)

NASA Astrophysics Data System (ADS)

Cappelli, Carlotta; Agnini, Claudia; Yamamoto, Yuhji

2017-04-01

The early-middle Eocene interval documents the shift from the warmest greenhouse conditions occurred during the Early Eocene Climatic Optimum (EECO, 52-50 Ma) to the beginning of the cooling phase which led to the Oligocene icehouse regime. This important transition is well expressed as a reversal in the global oxygen and carbonate isotope trends (Zachos et al., 2001). Moreover, this interval was a time of remarkable transformation in the marine biosphere. Communities of calcareous nannoplankton, marine calcifying algae at the base of the oceans food chain, experienced transient and permanent profound changes. Calcareous nannofossil are regarded as remarkable tools both in biostratigraphy and paleoecology, with several taxa that show different responses to changes in physical parameters of surface waters. Here, we aim to document calcareous nannoplankton assemblage changes across the early-middle Eocene transition, in order to upset the biostratigraphic framework and to increase comprehension of how phytoplankton communities responded to paleoenvironmental changes at that time. The sedimentary successions recovered at IODP Site U1410 (Exp. 342; 41˚ 19.6987'N; 49˚ 10.1995'W, Norris et al., 2012) on the Southeast Newfoundland Ridge (NW Atlantic) offer an expanded record of the early-middle Eocene interval that is marked by an increase in accumulation rate related to sedimentation of clay-rich nannofossil oozes. Quantitative analysis of calcareous nannofossil assemblages was conducted, encompassing calcareous nannofossil Zones NP12 -NP15 or CNE4-CNE10 (Martini, 1971; Agnini et al., 2014). The study interval records the appearance and proliferation of Noelaerhabdaceae family (i.e, Reticulofenestra/Dictyococcites group), which can be considered one of the most significant shifts in the assemblage structure of the Paleogene. This change was probably favored by modifications in surface water chemistry. The middle Eocene clay-rich sediments contain well preserved nannofossils, making this Site suitable for a comprehensive taxonomic revision of Nannotetrina and Chiasmolithus. Biohorizons related to species belonging to these two genera are used to mark middle Eocene biozone boundary, a better characterization of their taxonomy would thus improve their reliability as biostratigraphic tools. Furthermore, during the early middle Eocene a new evolutionary lineage, which includes S. kempii - S. perpendicularis- S. furcatholitoides morph. A - S. cuniculus - S. furcatholitoides morph. B occurred among sphenoliths. This plexus is characterized by progressive morphological changes which, if correctly identify, will allow for a very detailed subdivision of this interval. Even more interestingly, we would assess if there is any relationship between this evolutionary trend and the surrounding abiotic conditions. Agnini, C., Fornaciari, E., Raffi, I., Catanzariti, R., Pälike, H., Backman, J., and Rio, D., Newslett. Stratigr., 47, 131-181 (2014). Martini E., in: Farinacci, A. (Ed.), Proceedings 2nd International Conference Planktonic Microfossils Roma: Rome (Ed. Tecnosci.) 2, 739-785 (1971). Norris R.D., Wilson P.A., Blum P. and the IODP Expedition 342 Scientists, Proceedings of the Integrated Ocean Drilling Program, 342, 1-148 (2012). Zachos J., Pagani M., Sloan L., Thomas E., Billups K., Science, 292, 686-693 (2001).

Two-Year Stability and Change in Access to and Reasons for Lacking a Usual Source of Care Among Working-Age US Adults.

PubMed

Stransky, Michelle L

The objective of this study was to investigate usual source of care (USC) over time. A nationally representative sample of working-age adults in the Medical Expenditure Panel Survey (Panels 16 [2011-2012] and 17 [2012-2013]) was divided into 3 groups based on USC questions asked during 2 waves: (1) those having a USC during both waves (always USC), (2) those not having a USC during either wave (never USC), and (3) those who gained or lost a USC between the 2 waves (changed USC). The study examined the sociodemographic and health characteristics associated with these groups and the main reasons for not having a USC among those who never had a USC and those who changed their USC. Of the 10 792 adults in the analysis, a longitudinally weighted 18.8% reported that their USC changed during the period. Among adults in the changed USC group, those with private (odds ratio [OR] = 2.0, P = .001) or public (OR = 2.2, P = .001) health insurance and 1 (OR = 1.7, P = .04) or ≥2 (OR = 3.1, P = .02) chronic health conditions were more likely to transition to having a USC from not having a USC, compared with those who had no insurance and no chronic conditions. Compared with those in the changed USC group, those in the never USC group were more likely to report preferring not to have a USC (57.9% vs 80.1%, P < .001). Insurance and public health policies and programs are needed to reduce short-term changes in USC and increase the number of adults with ongoing, consistent USC.

Website Redesign: A Case Study.

PubMed

Wu, Jin; Brown, Janis F

2016-01-01

A library website redesign is a complicated and at times arduous task, requiring many different steps including determining user needs, analyzing past user behavior, examining other websites, defining design preferences, testing, marketing, and launching the site. Many different types of expertise are required over the entire process. Lessons learned from the Norris Medical Library's experience with the redesign effort may be useful to others undertaking a similar project.

Application of Nanoparticles/Nanowires and Carbon Nanotubes for Breast Cancer Research

DTIC Science & Technology

2005-09-01

References ALI : Please (see Fig. 8A, the current decreased from 5.94 pA to 1.43 jA). I. Ruoff, R. S. and Lorents, D. C. (1995), Cat-bon 33, 925-930...51,418-427. names. received only the PBS solution. When near infrared light 7. Dubertret, S. B., Norris, D. J., Noireaux, V., Brivanlou , A. H., about 800

Collecting to Win: ISR for Strategic Effect

DTIC Science & Technology

2014-02-13

Cryptologic History (Ft. George G. Meade , MD: National Security Agency, 1992), 1-143; Matthew M. Aid, The Secret Sentry: The Untold History of the National...United States Cryptologic History. Ft. George G. Meade , MD: National Security Agency, 1992. Norris, Pat. Spies in the Sky: Surveillance Satellites in...Submitted to the Faculty In Partial Fulfillment of the Graduation Requirements Advisor: Dr. Herbert L. Frandsen, Jr. 13 February 2014

Marine Mammal Acoustic Monitoring and Habitat Investigation, Southern California Offshore Region

DTIC Science & Technology

2009-06-01

whales , baleen whales , odontocetes, SCORE Range , offshore southern California, CalCOFI, blue whale , humpback whale , fin whale , sea surface temperature...they have been visually and acoustically detected off California during those times of year (Forney and Barlow 1998 ; Norris et al. 1999; Oleson 2005...whereas blue whales were only rarely detected in early winter and late spring (Oleson et al. 2007). In summer, whale

Can I Order a Burger at rnacdonalds.com? Visual Similarity Effects of Multi-Letter Combinations at the Early Stages of Word Recognition

ERIC Educational Resources Information Center

Marcet, Ana; Perea, Manuel

2018-01-01

Previous research has shown that early in the word recognition process, there is some degree of uncertainty concerning letter identity and letter position. Here, we examined whether this uncertainty also extends to the mapping of letter features onto letters, as predicted by the Bayesian Reader (Norris & Kinoshita, 2012). Indeed, anecdotal…

Does RBC Storage Age Effect Inflammation, Immune Function and Susceptibility to Transfusion Associated Microchimerism in Critically Ill Patients? Adverse Effects of RBC Storage in Critically Ill Patients

DTIC Science & Technology

2013-12-01

PROGRAM ELEMENT NUMBER 6. AUTHOR(S) Philip Spinella, M.D. 5d. PROJECT NUMBER Philip J. Norris , M.D.; Avani Shah, MPH 5e. TASK NUMBER Email...dysfunction syndrome , serious thrombotic events and nosocomial infections, and ICU and hospital length of stay. Prospective clinical studies investigating

Cecilia Payne-Gaposchkin, Henry Norris Russell Lecture: Fifty Years of Novae

NASA Astrophysics Data System (ADS)

Burbidge, E. M.

1999-05-01

It is easy to pick out my most memorable meeting of the AAS: the 149th meeting held in January, 1977, and hosted by the University of Hawaii, in Honolulu, HI. It was the meeting at which two traditions of the Society were broken, and we moved into the era of equal opportunity for women astronomers. Cecilia Payne-Gaposchkin received the highest award of the AAS: the Henry Norris Russell Lectureship. This award had never before been available to women, otherwise Cecilia would, years earlier, have been honored for the many achievements in her lifetime of renowned astronomical research. And I, the first woman to be elected President of the AAS, had the honor of presenting the illuminated scroll to Cecilia, and of introducing her on the platform where she delivered the Henry Norris Russell Prize Lecture, entitled ``Fifty Years of Novae"(1) . Cecilia opened by comparing the experience of young and old scientists in achieving exciting results from their research, and then led us through the history of the discoveries of and about some famous novae. She described the physical picture that emerged from studies of their light curves, their spectra, and the discovery of their binary nature. Three important tables were included, listing data on cataclysmic binaries (dwarf novae) and their link to the nova phenomenon in general. She recalled that she and Sergei Gaposchkin had hesitated between the names catastrophic and cataclysmic for the dwarf novae, and decided on the latter, from the dictionary definitions of those two terms: ``a cataclysm is a great and general flood" while a catastrophe ``is a final event". The nova phenomenon is recurrent, as are the dwarf novae, and both involve an outpouring of a flood of energy. She concluded by describing her 50 years' experience with novae as presenting ``the contemporary portrait of a nova", rather than a final picture, and by forecasting that the next 50 years of discovering and studying novae will be as full of surprises as the last. (1) Cecilia H. Payne-Gaposchkin, 1977, AJ, 82, 665.

Norrie disease pedigree carrying the novel mutation C65Y, predicted to disrupt the cystine knot growth factor motif, analyzed by RasI restriction digestion

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

Strasberg, P.M.; Liede, H.A.; Stein, T.

1994-09-01

Norrie disease (MIM 310600; ND) is an X-linked (Xp11.2-11.3) neurodevelopmental disorder characterized by congenital blindness, retinal dysplasia with pseudoglioma formation, and often associated with progressive mental retardation and deafness. The ND gene, comprised of 3 exons, codes for an evolutionarily conserved protein of 133 amino acids. We have analyzed 8 pedigrees segregating Norrie disease. Although microdeletions have been detected in several typical ND patients, Southern blot analysis with probes L1.28, MAO-A, MAO-B, TIMP-3.9X, pTak8, and M27{beta} failed to detect such deletions in these 8 ND pedigrees. With the cloning of the ND gene, PCR analysis of all 3 exons likewisemore » did not reveal any insertions or deletions. SSCP analysis ({sup 35}S-dNTP PCR) on PCR products of exon 3 showed a band shift for 1 patient. Repeat `cold` SSCP on minigels (3 inches x 4 inches) followed by liver staining was confirmatory. Direct sequencing revealed a G{r_arrow}A transition at nucleotide 610 corresponding to amino acid 65, changing Cys to Tyr. The mutation created an RsaI site, such that the uncut, normal, and mutant PCR products (using the same PCR primers) were 297 bp, 243 and 54 bp, and 177, 72 and 54 bp respectively. Affected males in the relevant pedigree had restricted PCR products of 177, 72 and 54 bp, carrier mothers 243, 177, 72, and 54 bp, and normals, including 30 unrelated individuals, 243 and 54 bp. Recent evidence indicates that the ND gene has a C-terminal domain homologous to that of TGF{beta}, thus identifying it as putative peptide growth factor, providing a monogenic disease model for the family of cystine knot growth factors. This is the first report of a mutation in Cys 2, critical for crosslinking to Cys 5 forming a disulphide bridge which holds the cystine knot growth factor tertiary structure together.« less

7 CFR 1.183 - Proceedings covered.

Code of Federal Regulations, 2010 CFR

2010-01-01

... Act of 1937 (7 U.S.C. 608c(15)(A)) Animal Health Protection Act, sections 10414 and 10415 (7 U.S.C... Information Act (7 U.S.C. 2912) Capper-Volstead Act (7 U.S.C. 292) Cotton Research and Promotion Act (7 U.S.C... Research and Promotion Act (7 U.S.C. 2620) Poultry Products Inspection Act (21 U.S.C. 455, 456, 457(d), 467...

42 CFR 137.290 - What additional provisions of law are related to NEPA and NHPA?

Code of Federal Regulations, 2012 CFR

2012-10-01

...) Archeological Resources Protection Act [16 U.S.C. 470aa]; (3) Clean Air Act [42 U.S.C. 7401]; (4) Clean Water Act [33 U.S.C. 1251]; (5) Coastal Barrier Improvement Act [42 U.S.C. 4028 and 16 U.S.C. Sec. 3501]; (6) Coastal Barrier Resources Act [16 U.S.C. 3501]; (7) Coastal Zone Management Act [16 U.S.C. 1451]; (8...

42 CFR 137.290 - What additional provisions of law are related to NEPA and NHPA?

Code of Federal Regulations, 2010 CFR

2010-10-01

...) Archeological Resources Protection Act [16 U.S.C. 470aa]; (3) Clean Air Act [42 U.S.C. 7401]; (4) Clean Water Act [33 U.S.C. 1251]; (5) Coastal Barrier Improvement Act [42 U.S.C. 4028 and 16 U.S.C. Sec. 3501]; (6) Coastal Barrier Resources Act [16 U.S.C. 3501]; (7) Coastal Zone Management Act [16 U.S.C. 1451]; (8...

42 CFR 137.290 - What additional provisions of law are related to NEPA and NHPA?

Code of Federal Regulations, 2013 CFR

2013-10-01

...) Archeological Resources Protection Act [16 U.S.C. 470aa]; (3) Clean Air Act [42 U.S.C. 7401]; (4) Clean Water Act [33 U.S.C. 1251]; (5) Coastal Barrier Improvement Act [42 U.S.C. 4028 and 16 U.S.C. Sec. 3501]; (6) Coastal Barrier Resources Act [16 U.S.C. 3501]; (7) Coastal Zone Management Act [16 U.S.C. 1451]; (8...

42 CFR 137.290 - What additional provisions of law are related to NEPA and NHPA?

Code of Federal Regulations, 2011 CFR

2011-10-01

...) Archeological Resources Protection Act [16 U.S.C. 470aa]; (3) Clean Air Act [42 U.S.C. 7401]; (4) Clean Water Act [33 U.S.C. 1251]; (5) Coastal Barrier Improvement Act [42 U.S.C. 4028 and 16 U.S.C. Sec. 3501]; (6) Coastal Barrier Resources Act [16 U.S.C. 3501]; (7) Coastal Zone Management Act [16 U.S.C. 1451]; (8...

42 CFR 137.290 - What additional provisions of law are related to NEPA and NHPA?

Code of Federal Regulations, 2014 CFR

2014-10-01

...) Archeological Resources Protection Act [16 U.S.C. 470aa]; (3) Clean Air Act [42 U.S.C. 7401]; (4) Clean Water Act [33 U.S.C. 1251]; (5) Coastal Barrier Improvement Act [42 U.S.C. 4028 and 16 U.S.C. Sec. 3501]; (6) Coastal Barrier Resources Act [16 U.S.C. 3501]; (7) Coastal Zone Management Act [16 U.S.C. 1451]; (8...

Department of Defense / General Services Administration / National...

Federal Register 2010, 2011, 2012, 2013, 2014

2010-12-20

... ADMINISTRATION (FAR) Final Rule Stage 492. FAR CASE 2006-005, HUBZONE PROGRAM REVISIONS Legal Authority: 40 USC 121(c); 10 USC ch 137; 42 USC 2473(c) Abstract: The Civilian Agency Acquisition Council and the... REPORTING PROCEDURES Legal Authority: 40 USC 121(c); 10 USC ch 137; 42 USC 2473(c) Abstract: The Civilian...

Marine Biotoxins: Laboratory Culture and Molecular Structure

DTIC Science & Technology

1989-10-18

associated with the human fish intoxication known as ciguatera , ciguatoxin and umitotoxin. and with palytoxin. Each of the three toxins presents a different...B. Current Status 1. Ciguatoxin The PI reviewed the status of ciguatera research at the Mycotoxins and Phycotoxins Symposium "n August, 1988 in...CITED BOMBER. J.W.. RUBIO. G.G. and NORRIS. D.R. (1989) Epiphytism of dinoflagellates associated with the disease ciguatera : substrate specificity and

Orcas in Puget Sound

DTIC Science & Technology

2007-01-01

Whales. Poster presented for the Society for Marine Mam- mal Meeting. Bain, D.E. 2002. A model linking energetic effects of whale watching to killer...P.L. Tyack, and H. Whitehead (eds.). Cetacean societies : field studies of dolphins and whales. University of Chicago Press, Chicago, IL. Baird, R.W...The feeding ecology of killer whales (Orcinus orca) in the Pacific Northwest. Pages 113-147 in K. Pryor and K.S. Norris (eds.). Dolphin societies

Linkage analysis of Norrie disease with an X-chromosomal ornithine aminotransferase locus.

PubMed

Bateman, J B; Kojis, T L; Cantor, R M; Heinzmann, C; Ngo, J T; Spence, M A; Inana, G; Kivlin, J D; Curtis, D; Sparkes, R S

1993-01-01

Norrie disease is a rare disease of newborn males caused by prenatal or perinatal retinal detachment, which may be associated with mental retardation, psychosis, and/or hearing loss. DXS7 (L1.28) and MAO A and B loci have been linked to the ND locus on the short arm of the X chromosome. Sequences homologous to OAT also have been mapped to the short arm of the X chromosome. We performed linkage analyses between the ND locus and one of the OAT-like clusters of sequences on the X chromosome (OATL1), using a ScaI RFLP in a ND family, and increased the previously calculated lod score (z) to over 3 (3.38; theta = 0.05). Similarly, we calculated a lod score of 4.06 (theta = 0.01) between the OATL1 and DXS7 loci. Alone, the OATL1 ScaI RFLP system is expected to be informative in 48% of females. If this system were used in combination with the DXS7 TaqI polymorphism, 71% of females would be informative for at least one of the markers and 21% would be informative for both. Because the OATL1 ScaI RFLP is a relatively common polymorphism, this system should be useful for the identification of ND carriers and affected male fetuses and newborns.

Non-conventional Frizzled ligands and Wnt receptors.

PubMed

Hendrickx, Marijke; Leyns, Luc

2008-05-01

The Wnt family of secreted signaling factors plays numerous roles in embryonic development and in stem cell biology. In the adult, Wnt signaling is involved in tissue homeostasis and mutations that lead to the overexpression of Wnt can be linked to cancer. Wnt signaling is transduced intracellularly by the Frizzled (Fzd) family of receptors. In the canonical pathway, accumulation of beta-catenin and the subsequent formation of a complex with T cell factors (TCF) or lymphoid enhancing factors (Lef) lead to target gene activation. The identification of Ryk as an alternative Wnt receptor and the discovery of the novel Fzd ligands Norrie disease protein (NDP) and R-Spondin, changed the traditional view of Wnts binding to Fzd receptors. Mouse R-Spondin cooperates with Wnt signaling and Low density lipoprotein (LDL) receptor related protein (LRP) to activate beta-catenin dependent gene expression and is involved in processes such as limb and placental development in the mouse. NDP is the product of the Norrie disease gene and controls vascular development in the retina, inner ear and in the female reproductive system during pregnancy. In this review a functional overview of the interactions of the different Wnt and non-Wnt ligands with the Fzd receptors is given as well as a survey of Wnts binding to Ryk and we discuss the biological significance of these interactions.

Novel mutation in TSPAN12 leads to autosomal recessive inheritance of congenital vitreoretinal disease with intra-familial phenotypic variability.

PubMed

Gal, Moran; Levanon, Erez Y; Hujeirat, Yasir; Khayat, Morad; Pe'er, Jacob; Shalev, Stavit

2014-12-01

Developmental malformations of the vitreoretinal vasculature are a heterogeneous group of conditions with various modes of inheritance, and include familial exudative vitreoretinopathy (FEVR), persistent fetal vasculature (PFV), and Norrie disease. We investigated a large consanguineous kindred with multiple affected individuals exhibiting variable phenotypes of abnormal vitreoretinal vasculature, consistent with the three above-mentioned conditions and compatible with autosomal recessive inheritance. Exome sequencing identified a novel c.542G > T (p.C181F) apparently mutation in the TSPAN12 gene that segregated with the ocular disease in the family. The TSPAN12 gene was previously reported to cause dominant and recessive FEVR, but has not yet been associated with other vitreoretinal manifestations. The intra-familial clinical variability caused by a single mutation in the TSPAN12 gene underscores the complicated phenotype-genotype correlation of mutations in this gene, and suggests that there are additional genetic and environmental factors involved in the complex process of ocular vascularization during embryonic development. Our study supports considering PFV, FEVR, and Norrie disease a spectrum of disorders, with clinical and genetic overlap, caused by mutations in distinct genes acting in the Norrin/β-catenin signaling pathway. © 2014 Wiley Periodicals, Inc.

Expression of the Norrie disease gene (Ndp) in developing and adult mouse eye, ear, and brain.

PubMed

Ye, Xin; Smallwood, Philip; Nathans, Jeremy

2011-01-01

The Norrie disease gene (Ndp) codes for a secreted protein, Norrin, that activates canonical Wnt signaling by binding to its receptor, Frizzled-4. This signaling system is required for normal vascular development in the retina and for vascular survival in the cochlea. In mammals, the pattern of Ndp expression beyond the retina is poorly defined due to the low abundance of Norrin mRNA and protein. Here, we characterize Ndp expression during mouse development by studying a knock-in mouse that carries the coding sequence of human placental alkaline phosphatase (AP) inserted at the Ndp locus (Ndp(AP)). In the CNS, Ndp(AP) expression is apparent by E10.5 and is dynamic and complex. The anatomically delimited regions of Ndp(AP) expression observed prenatally in the CNS are replaced postnatally by widespread expression in astrocytes in the forebrain and midbrain, Bergman glia in the cerebellum, and Müller glia in the retina. In the developing and adult cochlea, Ndp(AP) expression is closely associated with two densely vascularized regions, the stria vascularis and a capillary plexus between the organ of Corti and the spiral ganglion. These observations suggest the possibility that Norrin may have developmental and/or homeostatic functions beyond the retina and cochlea. Copyright © 2010 Elsevier B.V. All rights reserved.

Sequence analysis and transcript identification within 1.5 MB of DNA deleted together with the NDP and MAO genes in atypical Norrie disease patients presenting with a profound phenotype.

PubMed

Suárez-Merino, B; Bye, J; McDowall, J; Ross, M; Craig, I W

2001-06-01

Mutations at the Norrie disease gene locus, NDP, manifest in a broad range of defects. These range from a relatively mild, late-onset, exudative vitreoretinopathy to congenital blindness and sensorineural deafness combined, in some cases, with mental retardation. In addition, extensive deletions involving the NDP locus, located at Xp11.3, the adjacent monoamine oxidadase genes MAOA and MAOB, and additional material, result in a more severe pattern of symptoms. The phenotypes include all or some of the following; mental retardation, involuntary movements, hypertensive crises and hypogonadism. We extended an existing YAC contig to embrace the boundaries of three of the largest deletions and converted this into four PAC contigs. Computer analysis and experimental data have resulted in the identification of several putative loci, including a phosphatase inhibitor 2-like gene (dJ154.1) and a 250-bp sequence which resembles a homeobox domain (dA113.3), 1.2 Mb and 400 kb respectively from the MAO/NDP cluster. The pattern of expression of dJ154.1 suggests that it may represent an important factor contributing to the complex phenotypes of these deletion patients. Hum Mutat 17:523, 2001. Copyright 2001 Wiley-Liss, Inc.

Intrafamilial variability of the ocular phenotype in a Polish family with a missense mutation (A63D) in the Norrie disease gene.

PubMed

Zaremba, J; Feil, S; Juszko, J; Myga, W; van Duijnhoven, G; Berger, W

1998-09-01

To describe the phenotypic variability in a Polish Norrie disease (ND) family associated with the missense mutation A63D. A patient with spared vision from a Polish ND family underwent detailed ophthalmological examinations including slit-lamp biomicroscopy, ultrasound (USG), angiography, Goldmann kinetic visual field, and electroretinography (ERG). Mutation screening was carried out using the single-strand conformation polymorphism (SSCP) technique and subsequent DNA sequencing of the coding part of the ND gene. A mutation was detected (exon 3, A63D) in a large Polish family with 12 affected males, all but one presenting with classical ND symptoms. In one male, partially preserved vision was observed up to 40 years of age (distance acuity of the right eye 1/50 and left eye 2/50). Slit-lamp examination revealed remnants of a persistent primary vitreous and hyaloid artery. Upon angiography, the retina was vascularized within the posterior pole but not in the periphery. The ERG revealed pathological changes characteristic for chorioretinal degenerations. Within one family, individuals with identical sequence alterations in the ND gene can show remarkable phenotypic variability of the ocular symptoms. These findings indicate the involvement of additional factors (epigenetic or genetic) in ocular pathogenesis of ND.

Expression of the Norrie disease gene (Ndp) in developing and adult mouse eye, ear, and brain

PubMed Central

Ye, Xin; Smallwood, Philip; Nathans, Jeremy

2011-01-01

The Norrie disease gene (Ndp) codes for a secreted protein, Norrin, that activates canonical Wnt signaling by binding to its receptor, Frizzled-4. This signaling system is required for normal vascular development in the retina and for vascular survival in the cochlea. In mammals, the pattern of Ndp expression beyond the retina is poorly defined due to the low abundance of Norrin mRNA and protein. Here we characterize Ndp expression during mouse development by studying a knock-in mouse that carries the coding sequence of human placental alkaline phosphatase (AP) inserted at the Ndp locus (NdpAP). In the CNS, NdpAP expression is apparent by E10.5 and is dynamic and complex. The anatomically delimited regions of NdpAP expression observed prenatally in the CNS are replaced postnatally by widespread expression in astrocytes in the forebrain and midbrain, Bergman glia in the cerebellum, and Müller glia in the retina. In the developing and adult cochlea, NdpAP expression is closely associated with two densely vascularized regions, the stria vascularis and a capillary plexus between the organ of Corti and the spiral ganglion. These observations suggest the possibility that Norrin may have developmental and/or homeostatic functions beyond the retina and cochlea. PMID:21055480

Bethe, Oppenheimer, Teller and the Fermi Award: Norris Bradbury Speaks

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

Meade, Roger Allen

In 1956 the Enrico Fermi Presidential Award was established to recognize scientists, engineers, and science policymakers who gave unstintingly over their careers to advance energy science and technology. The first recipient was John von Neumann. .1 Among those scientists who were thought eligible for the award were Hans Bethe, J. Robert Oppenheimer, and Edward Teller. In 1959 Norris Bradbury was asked to comment on the relative merits of each these three men, whom he knew well from their affiliation with Los Alamos. Below is a reproduction of the letter Bradbury sent to Dr. Warren C. Johnson of the AEC’s Generalmore » Advisory Committee(GAC) containing his evaluation of each man. The letter might surprise those not accustomed to Bradbury’s modus operandi of providing very detailed and forthright answers to the AEC. The letter, itself, was found in cache of old microfilm. Whether because of the age of the microfilm or the quality of the filming process, portions of the letter are not legible. Where empty brackets appear, the word or words could not be read or deduced. Words appearing in brackets are guesses that appear, from the image, to be what was written. These guesses, of course, are just that – guesses.« less

Overproduction and partial purification of the Norrie disease gene product, norrin, from a recombinant baculovirus.

PubMed

Shastry, Barkur S; Trese, Michael T

2003-12-05

Abnormal vascularization of the peripheral retina and retinal detachment are common clinical characteristics of Norrie disease (ND), familial exudative vitreoretinopathy, Coats' disease, and retinopathy of prematurity. Although little is known about the molecular basis of these diseases, studies have shown that all of these diseases are associated with mutations in the ND gene. In spite of this, little is known about norrin, its molecular mechanism of action, and its functional relationship with the development of abnormal retinal vasculature. To obtain a large quantity of norrin for structural and functional studies, we have overproduced it in insect cells. For this purpose, a cDNA fragment (869 bp) was isolated from a human retinal cDNA library by amplification and was cloned into an expression vector. The purified plasmid was co-transfected with wild-type linearized Bac-N-Blue DNA into S. frugiperda Sf21 insect cells. The recombinant virus plaques were purified and clones were selected based on the level of recombinant protein expressed in Sf21 cells infected with a purified recombinant virus. From these, a high-titer stock was generated and subsequently used to prepare a fused protein on a large scale. The protein was partially purified by the process of immobilized metal affinity chromatography and the use of ion exchange chromatography

Biological characteristics of human-urine-derived stem cells: potential for cell-based therapy in neurology.

PubMed

Guan, Jun-Jie; Niu, Xin; Gong, Fei-Xiang; Hu, Bin; Guo, Shang-Chun; Lou, Yuan-Lei; Zhang, Chang-Qing; Deng, Zhi-Feng; Wang, Yang

2014-07-01

Stem cells in human urine have gained attention in recent years; however, urine-derived stem cells (USCs) are far from being well elucidated. In this study, we compared the biological characteristics of USCs with adipose-derived stem cells (ASCs) and investigated whether USCs could serve as a potential cell source for neural tissue engineering. USCs were isolated from voided urine with a modified culture medium. Through a series of experiments, we examined the growth rate, surface antigens, and differentiation potential of USCs, and compared them with ASCs. USCs showed robust proliferation ability. After serial propagation, USCs retained normal karyotypes. Cell surface antigen expression of USCs was similar to ASCs. With lineage-specific induction factors, USCs could differentiate toward the osteogenic, chondrogenic, adipogenic, and neurogenic lineages. To assess the ability of USCs to survive, differentiate, and migrate, they were seeded onto hydrogel scaffold and transplanted into rat brain. The results showed that USCs were able to survive in the lesion site, migrate to other areas, and express proteins that were associated with neural phenotypes. The results of our study demonstrate that USCs possess similar biological characteristics with ASCs and have multilineage differentiation potential. Moreover USCs can differentiate to neuron-like cells in rat brain. The present study shows that USCs are a promising cell source for tissue engineering and regenerative medicine.

50 CFR 10.1 - Purpose of regulations.

Code of Federal Regulations, 2012 CFR

2012-10-01

... wildlife: Lacey Act, 18 U.S.C. 42. Lacey Act Amendments of 1981, 16 U.S.C. 3371-3378. Migratory Bird Treaty Act, 16 U.S.C. 703-712. Bald and Golden Eagle Protection Act, 16 U.S.C. 668a-668d. Endangered Species Act of 1973, 16 U.S.C. 1531-1543. Tariff Classification Act of 1962, 19 U.S.C. 1202, [Schedule 1, Part...

50 CFR 10.1 - Purpose of regulations.

Code of Federal Regulations, 2011 CFR

2011-10-01

... wildlife: Lacey Act, 18 U.S.C. 42. Lacey Act Amendments of 1981, 16 U.S.C. 3371-3378. Migratory Bird Treaty Act, 16 U.S.C. 703-712. Bald and Golden Eagle Protection Act, 16 U.S.C. 668a-668d. Endangered Species Act of 1973, 16 U.S.C. 1531-1543. Tariff Classification Act of 1962, 19 U.S.C. 1202, [Schedule 1, Part...

50 CFR 10.1 - Purpose of regulations.

Code of Federal Regulations, 2013 CFR

2013-10-01

... wildlife: Lacey Act, 18 U.S.C. 42. Lacey Act Amendments of 1981, 16 U.S.C. 3371-3378. Migratory Bird Treaty Act, 16 U.S.C. 703-712. Bald and Golden Eagle Protection Act, 16 U.S.C. 668a-668d. Endangered Species Act of 1973, 16 U.S.C. 1531-1543. Tariff Classification Act of 1962, 19 U.S.C. 1202, [Schedule 1, Part...

50 CFR 10.1 - Purpose of regulations.

Code of Federal Regulations, 2014 CFR

2014-10-01

... wildlife: Lacey Act, 18 U.S.C. 42. Lacey Act Amendments of 1981, 16 U.S.C. 3371-3378. Migratory Bird Treaty Act, 16 U.S.C. 703-712. Bald and Golden Eagle Protection Act, 16 U.S.C. 668a-668d. Endangered Species Act of 1973, 16 U.S.C. 1531-1543. Tariff Classification Act of 1962, 19 U.S.C. 1202, [Schedule 1, Part...

50 CFR 10.1 - Purpose of regulations.

Code of Federal Regulations, 2010 CFR

2010-10-01

... wildlife: Lacey Act, 18 U.S.C. 42. Lacey Act Amendments of 1981, 16 U.S.C. 3371-3378. Migratory Bird Treaty Act, 16 U.S.C. 703-712. Bald and Golden Eagle Protection Act, 16 U.S.C. 668a-668d. Endangered Species Act of 1973, 16 U.S.C. 1531-1543. Tariff Classification Act of 1962, 19 U.S.C. 1202, [Schedule 1, Part...

Exosomal DMBT1 from human urine-derived stem cells facilitates diabetic wound repair by promoting angiogenesis.

PubMed

Chen, Chun-Yuan; Rao, Shan-Shan; Ren, Lu; Hu, Xiong-Ke; Tan, Yi-Juan; Hu, Yin; Luo, Juan; Liu, Yi-Wei; Yin, Hao; Huang, Jie; Cao, Jia; Wang, Zhen-Xing; Liu, Zheng-Zhao; Liu, Hao-Ming; Tang, Si-Yuan; Xu, Ran; Xie, Hui

2018-01-01

Chronic non-healing wounds represent one of the most common complications of diabetes and need advanced treatment strategies. Exosomes are key mediators of cell paracrine action and can be directly utilized as therapeutic agents for tissue repair and regeneration. Here, we explored the effects of exosomes from human urine-derived stem cells (USC-Exos) on diabetic wound healing and the underlying mechanism. Methods: USCs were characterized by flow cytometry and multipotent differentiation potential analyses. USC-Exos were isolated from the conditioned media of USCs and identified by transmission electron microscopy and flow cytometry. A series of functional assays in vitro were performed to assess the effects of USC-Exos on the activities of wound healing-related cells. Protein profiles in USC-Exos and USCs were examined to screen the candidate molecules that mediate USC-Exos function. The effects of USC-Exos on wound healing in streptozotocin-induced diabetic mice were tested by measuring wound closure rates, histological and immunofluorescence analyses. Meanwhile, the role of the candidate protein in USC-Exos-induced regulation of angiogenic activities of endothelial cells and diabetic wound healing was assessed. Results: USCs were positive for CD29, CD44, CD73 and CD90, but negative for CD34 and CD45. USCs were able to differentiate into osteoblasts, adipocytes and chondrocytes. USC-Exos exhibited a cup- or sphere-shaped morphology with a mean diameter of 51.57 ± 2.93 nm and positive for CD63 and TSG101. USC-Exos could augment the functional properties of wound healing-related cells including the angiogenic activities of endothelial cells. USC-Exos were enriched in the proteins that are involved in regulation of wound healing-related biological processes. Particularly, a pro-angiogenic protein called deleted in malignant brain tumors 1 (DMBT1) was highly expressed in USC-Exos. Further functional assays showed that DMBT1 protein was required for USC-Exos-induced promotion of angiogenic responses of cultured endothelial cells, as well as angiogenesis and wound healing in diabetic mice. Conclusion: Our findings suggest that USC-Exos may represent a promising strategy for diabetic soft tissue wound healing by promoting angiogenesis via transferring DMBT1 protein.

12 CFR 263.65 - Civil penalty inflation adjustments.

Code of Federal Regulations, 2014 CFR

2014-01-01

...) First tier—$7,500. (ii) Second tier—$37,500. (iii) Third tier—$1,425,000. (3) 12 U.S.C. 1820(k)(6)(A)(ii...) 12 U.S.C. 1467a(i)(2)—$32,500. (ii) 12 U.S.C. 1467a(i)(3)—$32,500. (12) 12 U.S.C. 1467a(r): (i) 12 U.S.C. 1467a(r)(1)—$3,200. (ii) 12 U.S.C. 1467a(r)(2)—$32,500. (iii) 12 U.S.C. 1467a(r)(3)—$1,425,000...

12 CFR 263.65 - Civil penalty inflation adjustments.

Code of Federal Regulations, 2013 CFR

2013-01-01

...) First tier—$7,500. (ii) Second tier—$37,500. (iii) Third tier—$1,425,000. (3) 12 U.S.C. 1820(k)(6)(A)(ii...) 12 U.S.C. 1467a(i)(2)—$32,500. (ii) 12 U.S.C. 1467a(i)(3)—$32,500. (12) 12 U.S.C. 1467a(r): (i) 12 U.S.C. 1467a(r)(1)—$3,200. (ii) 12 U.S.C. 1467a(r)(2)—$32,500. (iii) 12 U.S.C. 1467a(r)(3)—$1,425,000...

Parent and Child Usual Source of Care and Children’s Receipt of Health Care Services

PubMed Central

DeVoe, Jennifer E.; Tillotson, Carrie J.; Wallace, Lorraine S.; Angier, Heather; Carlson, Matthew J.; Gold, Rachel

2011-01-01

PURPOSE In the United States, children who have a usual source of care (USC) have better access to health care than those who do not, but little is known about how parental USC affects children’s access. We examined the association between child and parent USC patterns and children’s access to health care services. METHODS We undertook a secondary analysis of nationally representative, cross-sectional data from children participating in the 2002–2007 Medical Expenditure Panel Survey (n = 56,302). We assessed 10 outcome measures: insurance coverage gaps, no doctor visits in the past year, less than yearly dental visits, unmet medical and prescription needs, delayed care, problems getting care, and unmet preventive counseling needs regarding healthy eating, regular exercise, car safety devices, and bicycle helmets. RESULTS Among children, 78.6% had a USC and at least 1 parent with a USC, whereas 12.4% had a USC but no parent USC. Children with a USC but no parent USC had a higher likelihood of several unmet needs, including an insurance coverage gap (adjusted risk ratio [aRR] 1.33; 95% confidence interval [CI], 1.21–1.47), an unmet medical or prescription need (aRR 1.70; 95% CI 1.09–2.65), and no yearly dental visits (aRR 1.12; 95% CI 1.06–1.18), compared with children with a USC whose parent(s) had a USC. CONCLUSIONS Among children with a USC, having no parent USC was associated with a higher likelihood of reporting unmet needs when compared with children whose parent(s) had a USC. Policy reforms should ensure access to a USC for all family members. PMID:22084261

Parent and child usual source of care and children's receipt of health care services.

PubMed

DeVoe, Jennifer E; Tillotson, Carrie J; Wallace, Lorraine S; Angier, Heather; Carlson, Matthew J; Gold, Rachel

2011-01-01

PURPOSE In the United States, children who have a usual source of care (USC) have better access to health care than those who do not, but little is known about how parental USC affects children's access. We examined the association between child and parent USC patterns and children's access to health care services. METHODS We undertook a secondary analysis of nationally representative, cross-sectional data from children participating in the 2002-2007 Medical Expenditure Panel Survey (n = 56,302). We assessed 10 outcome measures: insurance coverage gaps, no doctor visits in the past year, less than yearly dental visits, unmet medical and prescription needs, delayed care, problems getting care, and unmet preventive counseling needs regarding healthy eating, regular exercise, car safety devices, and bicycle helmets. RESULTS Among children, 78.6% had a USC and at least 1 parent with a USC, whereas 12.4% had a USC but no parent USC. Children with a USC but no parent USC had a higher likelihood of several unmet needs, including an insurance coverage gap (adjusted risk ratio [aRR] 1.33; 95% confidence interval [CI], 1.21-1.47), an unmet medical or prescription need (aRR 1.70; 95% CI 1.09-2.65), and no yearly dental visits (aRR 1.12; 95% CI 1.06-1.18), compared with children with a USC whose parent(s) had a USC. CONCLUSIONS Among children with a USC, having no parent USC was associated with a higher likelihood of reporting unmet needs when compared with children whose parent(s) had a USC. Policy reforms should ensure access to a USC for all family members.

The Experience of Community in Canadian Military Families: A Female Partners’ Perspective

DTIC Science & Technology

2006-04-01

note how the changes in technology have improved their quality of life , especially during deployments and long work -related separations. This...community would benefit also from working with support agencies to create the kinds of policy that will encourage the growth of just such a community...1992). Alternative paradigms in family life education. Family Relations,41 12-17. Norris, D. (2001). Working them out… working them in: Ideology and

A Historical Perspective on Light Infantry

DTIC Science & Technology

1987-01-01

which were scarce) expecting the enemy to use them in building fires. In another case, the CCF draped a large wire entanglement across a village...Jungle Frontier: 92 Special Air Service Regiment in the Borneo Campaign, 1963-1966 (London: Arms and Armour Press, 1985), 65, 57. Lieutenant Colonel P. E...1963-1966. London: Arms and Armour Press, 1985. Foxley-Norris, C. N., Air Vice Marshal, "Air Aspects of Operations Against ’Confrontation.’ " In

Time-Resolved Electronic Relaxation Processes in Self-Organized Quantum Dots

DTIC Science & Technology

2005-05-16

in a quantum dot infrared photodetector ,” paper CthM11, presented at CLEO, Baltimore, 2003. K. Kim, T. Norris, J. Singh, P. Bhattacharya...nanostructures have been equally spectacular. Following the development of quantum-well infrared photodetectors in the late 1980’s and early 90’s...4]. The quantum cascade laser is of course the best known of the new devices, as it constitutes an entirely new concept in semiconductor laser

Accurate Virus Quantitation Using a Scanning Transmission Electron Microscopy (STEM) Detector in a Scanning Electron Microscope

DTIC Science & Technology

2017-06-29

Accurate Virus Quantitation Using a Scanning Transmission Electron Microscopy (STEM) Detector in a Scanning Electron Microscope Candace D Blancett1...L Norris2, Cynthia A Rossi4 , Pamela J Glass3, Mei G Sun1,* 1 Pathology Division, United States Army Medical Research Institute of Infectious...Diseases (USAMRIID), 1425 Porter Street, Fort Detrick, Maryland, 21702 2Biostatistics Division, United States Army Medical Research Institute of

Critical Information Literacy as Core Skill for Lifelong STEM Learning in the 21st Century: Reflections on the Desirability and Feasibility for Widespread Science Media Education

ERIC Educational Resources Information Center

Storksdieck, Martin

2016-01-01

Grace Reid and the late Stephen Norris argue in this issue the urgent need for widespread Science Media Education (SME) as an integral part of formal and informal science education. SME is to achieve two goals: First, allow learners to critically evaluate any media as a source for scientific information by understanding the socio-economic and…

Battle of Britain Toolbook

DTIC Science & Technology

1995-05-01

determine how lessons therefrom have future application. The British author, Group Captain A.G.B. Vallance , addresses British national defense policy in...of Air Force History, 1983. Estes, Richard H. "Guilio Douhet: More on Target Than He Knew," Airpower Journal. IV-4:68-78 (Winter 1990). Foxley-Norris...Co., Inc., 1971. Janes William H. "Dunkirk: A German Decision" Armour. LXXXIII-3:19-22 (May-June 1974). Kaplan, Philip and Richard Collier. Their

A survey of computer search service costs in the academic health sciences library.

PubMed Central

Shirley, S

1978-01-01

The Norris Medical Library, University of Southern California, has recently completed an extensive survey of costs involved in the provision of computer search services beyond vendor charges for connect time and printing. In this survey costs for such items as terminal depreciation, repair contract, personnel time, and supplies are analyzed. Implications of this cost survey are discussed in relation to planning and price setting for computer search services. PMID:708953

Techniques for Reaeration of Hydropower Releases.

DTIC Science & Technology

1983-02-01

peak production from air induction through the baffle ring. The other aeration technique at Norris required modifications to the vacuum-breaker system...of Gas Tracers for Reaeration," Jour. Environ. Div., Proc. Amer. Soc. Civil Engr., 104, 215, April. Rathbun, R. E., 1979, "Estimating the Gas and Dye ...or dissolved in the water, and--last but not least--by the decomposition of bottom mud and by oxidation of the decomposition products stirred up out

Durability of Flexible Ureteroscopes: A Prospective Evaluation of Longevity, the Factors that Affect it, and Damage Mechanisms.

PubMed

Legemate, Jaap D; Kamphuis, Guido M; Freund, Jan Erik; Baard, Joyce; Zanetti, Stefano P; Catellani, Michele; Oussoren, Harry W; de la Rosette, Jean J

2018-03-10

Flexible ureteroscopy is an established treatment modality for evaluating and treating abnormalities in the upper urinary tract. Reusable ureteroscope (USC) durability is a significant concern. To evaluate the durability of the latest generation of digital and fiber optic reusable flexible USCs and the factors affecting it. Six new flexible USCs from Olympus and Karl Storz were included. The primary endpoint for each USC was its first repair. Data on patient and treatment characteristics, accessory device use, ureteroscopy time, image quality, USC handling, disinfection cycles, type of damage, and deflection loss were collected prospectively. Ureteroscopy. USC durability was measured as the total number of uses and ureteroscopy time before repair. USC handling and image quality were scored. After every procedure, maximal ventral and dorsal USC deflection were documented on digital images. A total of 198 procedures were performed. The median number of procedures was 27 (IQR 16-48; 14h) for the six USCs overall, 27 (IQR 20-56; 14h) for the digital USCs, and 24 (range 10-37; 14h) for the fiber optic USCs. Image quality remained high throughout the study for all six USCs. USC handling and the range of deflection remained good under incremental use. Damage to the distal part of the shaft and shaft coating was the most frequent reason for repair, and was related to intraoperative manual forcing. A limitation of this study is its single-center design. The durability of the latest reusable flexible USCs in the current study was limited to 27 uses (14h). Damage to the flexible shaft was the most important limitation to the durability of the USCs evaluated. Prevention of intraoperative manual forcing of flexible USCs maximizes their overall durability. Current flexible ureteroscopes proved to be durable. Shaft vulnerability was the most important limiting factor affecting durability. Copyright © 2018 European Association of Urology. Published by Elsevier B.V. All rights reserved.

A medical home versus temporary housing: the importance of a stable usual source of care.

PubMed

DeVoe, Jennifer E; Saultz, John W; Krois, Lisa; Tillotson, Carrie J

2009-11-01

Little is known about how the stability of a usual source of care (USC) affects access to care. We examined the prevalence of USC changes among low-income children and how these changes were associated with unmet health care need. We conducted a cross-sectional survey of Oregon's food stamp program in 2005. We analyzed primary data from 2681 surveys and then weighted results to 84087 families, adjusting for oversampling and nonresponse. We then ascertained the percentage of children in the Oregon population who had ever changed a USC for insurance reasons, which characteristics were associated with USC change, and how USC change was associated with unmet need. We also conducted a posthoc analysis of data from the Medical Expenditure Panel Survey to confirm similarities between the Oregon sample and a comparable national sample. Children without a USC in the Oregon population had greater odds of reporting an unmet health care need than those with a USC. This pattern was similar in national estimates. Among the Oregon sample, 23% had changed their USC because of insurance reasons, and 10% had no current USC. Compared with children with a stable USC, children who had changed their USC had greater odds of reporting unmet medical need, unmet prescription need, delayed care, unmet dental need, and unmet counseling need. This study highlights the importance of ensuring stability with a USC. Moving low-income children into new medical homes could disturb existing USC relationships, thereby merely creating "temporary housing."

Exosomal DMBT1 from human urine-derived stem cells facilitates diabetic wound repair by promoting angiogenesis

PubMed Central

Chen, Chun-Yuan; Rao, Shan-Shan; Ren, Lu; Hu, Xiong-Ke; Tan, Yi-Juan; Hu, Yin; Luo, Juan; Liu, Yi-Wei; Yin, Hao; Huang, Jie; Cao, Jia; Wang, Zhen-Xing; Liu, Zheng-Zhao; Liu, Hao-Ming; Tang, Si-Yuan; Xu, Ran; Xie, Hui

2018-01-01

Chronic non-healing wounds represent one of the most common complications of diabetes and need advanced treatment strategies. Exosomes are key mediators of cell paracrine action and can be directly utilized as therapeutic agents for tissue repair and regeneration. Here, we explored the effects of exosomes from human urine-derived stem cells (USC-Exos) on diabetic wound healing and the underlying mechanism. Methods: USCs were characterized by flow cytometry and multipotent differentiation potential analyses. USC-Exos were isolated from the conditioned media of USCs and identified by transmission electron microscopy and flow cytometry. A series of functional assays in vitro were performed to assess the effects of USC-Exos on the activities of wound healing-related cells. Protein profiles in USC-Exos and USCs were examined to screen the candidate molecules that mediate USC-Exos function. The effects of USC-Exos on wound healing in streptozotocin-induced diabetic mice were tested by measuring wound closure rates, histological and immunofluorescence analyses. Meanwhile, the role of the candidate protein in USC-Exos-induced regulation of angiogenic activities of endothelial cells and diabetic wound healing was assessed. Results: USCs were positive for CD29, CD44, CD73 and CD90, but negative for CD34 and CD45. USCs were able to differentiate into osteoblasts, adipocytes and chondrocytes. USC-Exos exhibited a cup- or sphere-shaped morphology with a mean diameter of 51.57 ± 2.93 nm and positive for CD63 and TSG101. USC-Exos could augment the functional properties of wound healing-related cells including the angiogenic activities of endothelial cells. USC-Exos were enriched in the proteins that are involved in regulation of wound healing-related biological processes. Particularly, a pro-angiogenic protein called deleted in malignant brain tumors 1 (DMBT1) was highly expressed in USC-Exos. Further functional assays showed that DMBT1 protein was required for USC-Exos-induced promotion of angiogenic responses of cultured endothelial cells, as well as angiogenesis and wound healing in diabetic mice. Conclusion: Our findings suggest that USC-Exos may represent a promising strategy for diabetic soft tissue wound healing by promoting angiogenesis via transferring DMBT1 protein. PMID:29556344

Human Urine Derived Stem Cells in Combination with β-TCP Can Be Applied for Bone Regeneration.

PubMed

Guan, Junjie; Zhang, Jieyuan; Li, Haiyan; Zhu, Zhenzhong; Guo, Shangchun; Niu, Xin; Wang, Yang; Zhang, Changqing

2015-01-01

Bone tissue engineering requires highly proliferative stem cells that are easy to isolate. Human urine stem cells (USCs) are abundant and can be easily harvested without using an invasive procedure. In addition, in our previous studies, USCs have been proved to be able to differentiate into osteoblasts, chondrocytes, and adipocytes. Therefore, USCs may have great potential and advantages to be applied as a cell source for tissue engineering. However, there are no published studies that describe the interactions between USCs and biomaterials and applications of USCs for bone tissue engineering. Therefore, the objective of the present study was to evaluate the interactions between USCs with a typical bone tissue engineering scaffold, beta-Tricalcium Phosphate (β-TCP), and to determine whether the USCs seeded onto β-TCP scaffold can promote bone regeneration in a segmental femoral defect of rats. Primary USCs were isolated from urine and seeded on β-TCP scaffolds. Results showed that USCs remained viable and proliferated within β-TCP. The osteogenic differentiation of USCs within the scaffolds was demonstrated by increased alkaline phosphatase activity and calcium content. Furthermore, β-TCP with adherent USCs (USCs/β-TCP) were implanted in a 6-mm critical size femoral defect of rats for 12 weeks. Bone regeneration was determined using X-ray, micro-CT, and histologic analyses. Results further demonstrated that USCs in the scaffolds could enhance new bone formation, which spanned bone defects in 5 out of 11 rats while β-TCP scaffold alone induced modest bone formation. The current study indicated that the USCs can be used as a cell source for bone tissue engineering as they are compatible with bone tissue engineering scaffolds and can stimulate the regeneration of bone in a critical size bone defect.

Disparity of Chlorine to Fluorine Concentration Ratios Between Thermal Waters and Rocks of Yellowstone National Park, USA

NASA Astrophysics Data System (ADS)

McConville, E. G.; Szymanski, M. E.; Hurwitz, S.; Lowenstern, J. B.; Hayden, L. A.

2016-12-01

Low chlorine to fluorine concentration ratios (Cl/F) of 0.5 by weight are observed in Yellowstone rhyolites within glass inclusions and erupted rhyolitic glass. In contrast, Yellowstone thermal waters have Cl/F of >10 and Cl/F of waters at Norris Geyser Basin can exceed 100. Similar Cl/F have been observed in other volcanic hydrothermal systems (e.g., Lassen, Long Valley Caldera). The goal of this study is to identify fluorine-bearing minerals that could remove a substantial amount of F from the hydrothermal fluids within the Yellowstone caldera and in the Norris Geyser Basin near the northern margin of the caldera. We used a scanning electron microscope (SEM) to study thin sections from core samples obtained during research drilling by the USGS in the 1960s. The Y-2 well (Lower Geyser Basin) penetrated mostly Plateau Rhyolites ( 0.15 Ma) and Y-7 and Y-8 wells (Upper Geyser Basin) penetrated glacial sandstones and conglomerates, underlain by the Biscuit Basin flow ( 0.5 Ma). The thin sections from Y-12 in the Norris Geyser Basin are all from the Lava Creek Tuff. Fluorine-bearing minerals are found in all drill cores. Fluorite is present in Y-2 at a depth of 153 m, in Y-7 at 65m, and in Y-12 at 276 m. Fluoroapatite first appears in the Biscuit Basin flow at 60 m in Y-7 and 59 m in Y-8. Rare earth fluorocarbonates, such as bastnaesite (Ce,La,Y)CO3F and/or parisite Ca(Ce,La)2(CO3)3F2, are predominantly found in Y-12 at depths >276 m. Our estimated abundances of these fluorine-bearing minerals are at least 2 orders of magnitude less than required to substantially affect the Cl/F ratio in thermal waters. Fluorine-bearing minerals may be more abundant at greater depth. Another possible explanation is that the fluorite is too fine-grained to be identified by SEM. Finally, the high Cl/F in thermal waters could be explained by the ascent of Cl-rich fluid from a cooling magma body or from older crustal rocks that underlie the caldera.

Letter - Reply: Meteors in Australian Aboriginal Dreamings

NASA Astrophysics Data System (ADS)

Hamacher, Duane W.

2011-06-01

In response to the letter by Gorelli (2010) about Hamacher & Norris (2010), he is quite right about Aboriginal people witnessing impact events in Australia. There are several oral traditions regarding impact sites, some of which were probably witnessed, as Gorelli pointed out. The Henbury craters he mentions, with a young age of only ∼ 4200 years, have oral traditions that seem to describe a cosmic impact, including an aversion to drinking water that collects in the craters in fear that the fire-devil (which came from the sun, according to an Elder) would rain iron in them again. Other impact sites, such as Gosse's Bluff crater (Tnorala in the Arrernte language) and Wolfe Creek crater (Kandimalal in the Djaru language) have associated impact stories, despite their old ages (142 Ma and ∼0.3 Ma, respectively). In addition, many fireball and airburst events are described in Aboriginal oral traditions, a number of which seem to indicate impact events that are unknown to Western science. I have published a full treatise of meteorite falls and impact events in Australian Aboriginal culture that I would like to bring to the attention of Gorelli and WGN readers (Hamacher & Norris, 2009). Although our paper was published in the 2009 volume of Archaeoastronomy, it did not appear in print until just recently, which is probably why it has gone unnoticed. Recent papers describing the association between meteorites and Aboriginal cosmology (Hamacher, 2011) and comets in Aboriginal culture (Hamacher & Norris, 2011) have also been published, and would likely be of interest to WGN readers. I heartily agree with Gorelli that oral traditions are fast disappearing, taking with them a wealth of information about not only that peoples' culture, but also about past geologic and astronomical events, such as meteorite falls and cosmic impacts (a branch of the growing field of Geomythology). There is an old saying that "when a man dies, a library goes with him". This is certainly the case in Australia, and along with Gorelli, I encourage WGN readers to get involved in studying meteoritic events in oral traditions. There is a lot of information regarding meteoritical events and phenomena in the literature that is still waiting to be collected and analysed ...and I applaud McBeath for his pursuit to publish this material through the successful Meteor Beliefs Project!

48 CFR 215.403-1 - Prohibition on obtaining cost or pricing data (10 U.S.C. 2306a and 41 U.S.C. 254b).

Code of Federal Regulations, 2010 CFR

2010-10-01

... cost or pricing data (10 U.S.C. 2306a and 41 U.S.C. 254b). 215.403-1 Section 215.403-1 Federal... METHODS AND CONTRACT TYPES CONTRACTING BY NEGOTIATION Contract Pricing 215.403-1 Prohibition on obtaining cost or pricing data (10 U.S.C. 2306a and 41 U.S.C. 254b). (b) Exceptions to cost or pricing data...

12 CFR 263.65 - Civil penalty inflation adjustments.

Code of Federal Regulations, 2012 CFR

2012-01-01

... tier—$7,500. (ii) Second tier—$37,500. (iii) Third tier—$1,375,000. (3) 12 U.S.C. 1820(k)(6)(A)(ii...) 12 U.S.C. 1467a(i)(3)-$32,500. (12) 12 U.S.C. 1467a(r): (i) 12 U.S.C. 1467a(r)(1)-$ 2,200. (ii) 12 U.S.C. 1467a(r)(2)-$32,500. (iii) 12 U.S.C. 1467a(r)(3)-$1,375,000. [73 FR 58032, Oct. 6, 2008, as...

49 CFR 1105.1 - Purpose.

Code of Federal Regulations, 2012 CFR

2012-10-01

... decisionmaking process pursuant to the National Environmental Policy Act, 42 U.S.C. 4332; the Energy Policy and Conservation Act, 42 U.S.C. 6362(b); and related laws, including the National Historic Preservation Act, 16 U.S.C. 470f, the Coastal Zone Management Act, 16 U.S.C. 1451, and the Endangered Species Act, 16 U.S.C...

49 CFR 1105.1 - Purpose.

Code of Federal Regulations, 2013 CFR

2013-10-01

... decisionmaking process pursuant to the National Environmental Policy Act, 42 U.S.C. 4332; the Energy Policy and Conservation Act, 42 U.S.C. 6362(b); and related laws, including the National Historic Preservation Act, 16 U.S.C. 470f, the Coastal Zone Management Act, 16 U.S.C. 1451, and the Endangered Species Act, 16 U.S.C...

49 CFR 1105.1 - Purpose.

Code of Federal Regulations, 2011 CFR

2011-10-01

... decisionmaking process pursuant to the National Environmental Policy Act, 42 U.S.C. 4332; the Energy Policy and Conservation Act, 42 U.S.C. 6362(b); and related laws, including the National Historic Preservation Act, 16 U.S.C. 470f, the Coastal Zone Management Act, 16 U.S.C. 1451, and the Endangered Species Act, 16 U.S.C...

49 CFR 1105.1 - Purpose.

Code of Federal Regulations, 2014 CFR

2014-10-01

... decisionmaking process pursuant to the National Environmental Policy Act, 42 U.S.C. 4332; the Energy Policy and Conservation Act, 42 U.S.C. 6362(b); and related laws, including the National Historic Preservation Act, 16 U.S.C. 470f, the Coastal Zone Management Act, 16 U.S.C. 1451, and the Endangered Species Act, 16 U.S.C...

49 CFR 1105.1 - Purpose.

Code of Federal Regulations, 2010 CFR

2010-10-01

... decisionmaking process pursuant to the National Environmental Policy Act, 42 U.S.C. 4332; the Energy Policy and Conservation Act, 42 U.S.C. 6362(b); and related laws, including the National Historic Preservation Act, 16 U.S.C. 470f, the Coastal Zone Management Act, 16 U.S.C. 1451, and the Endangered Species Act, 16 U.S.C...

The Role of the Caspase-8 Inhibitor FLIP in Androgen-Withdrawal Induced Death of Prostate Epithelium

DTIC Science & Technology

2007-01-01

cells. Cancer Res 2000;60:2384-9. 20. Voelkel-Johnson C, King DL, Norris JS. Resistance of prostate cancer cells to soluble TNF-related apoptosis...Mukhopadhyay A, Bueso-Ramos C, Chatterjee D, Pantazis P, Aggarwal BB. Curcumin downregulates cell survival mechanisms in human prostate cancer cell...ethanol. LY294002 (Cayman) was dissolved in Androgens and FOXO3a regulate FLIP page 7 dimethylsulfoxide (DMSO). Soluble recombinant human TRAIL (Biomol

Joint Force Quarterly. Issue 67, 4th Quarter, October 2012

DTIC Science & Technology

2012-10-01

a penalty for failing to comply with a collection of information if it does not display a currently valid OMB control number. 1. REPORT DATE 2012 2...Defense Douglas N. Hime Naval War College Mark H. Jacobsen Marine Corps Command and Staff College Daniel T. Kuehl Information Resources Management...Norris Air Command and Staff College Col Mark Pizzo, USMC (Ret.) National War College James A . Schear Office of the Secretary of Defense LtGen Bernard

The Operational Capability of the American Expeditionary Forces in the World War

DTIC Science & Technology

2014-12-04

attend the Advance Military Studies Program, COLs Michael Getchell, John Marr, and John Norris . Finally, to my college mentor, Dario Lorenzetti, killed...the United States Army and the War Department was selecting a general to lead the American Army in France. Since the end of the Spanish -American...formation since the end of the Spanish -American War. This proved to be a great challenge for the American Army in finding leaders to command the large

The Deaf Man and the World; Work - Love - Worship - Play. Proceedings of National Forum II, Council of Organizations Serving the Deaf (New Orleans, Louisiana, February 19-22, 1969).

ERIC Educational Resources Information Center

Jones, Ray L., Ed.

Papers and speeches concern the interaction of the deaf to a hearing world. Areas considered are the self concept of the deaf by Robert G. Sanderson, the problem of employment by Arthur G. Norris, underemployment by Abraham Stahler, the relationship of the deaf to his hearing family by Rosslyn Gaines Suchman, and personal counseling by Richard K.…

Linkage analysis of Norrie disease with an X-chromosomal ornithine aminotransferase locus.

PubMed Central

Bateman, J B; Kojis, T L; Cantor, R M; Heinzmann, C; Ngo, J T; Spence, M A; Inana, G; Kivlin, J D; Curtis, D; Sparkes, R S

1993-01-01

Norrie disease is a rare disease of newborn males caused by prenatal or perinatal retinal detachment, which may be associated with mental retardation, psychosis, and/or hearing loss. DXS7 (L1.28) and MAO A and B loci have been linked to the ND locus on the short arm of the X chromosome. Sequences homologous to OAT also have been mapped to the short arm of the X chromosome. We performed linkage analyses between the ND locus and one of the OAT-like clusters of sequences on the X chromosome (OATL1), using a ScaI RFLP in a ND family, and increased the previously calculated lod score (z) to over 3 (3.38; theta = 0.05). Similarly, we calculated a lod score of 4.06 (theta = 0.01) between the OATL1 and DXS7 loci. Alone, the OATL1 ScaI RFLP system is expected to be informative in 48% of females. If this system were used in combination with the DXS7 TaqI polymorphism, 71% of females would be informative for at least one of the markers and 21% would be informative for both. Because the OATL1 ScaI RFLP is a relatively common polymorphism, this system should be useful for the identification of ND carriers and affected male fetuses and newborns. PMID:7908152

33 CFR 27.3 - Penalty Adjustment Table.

Code of Federal Regulations, 2010 CFR

2010-07-01

... Manufacture 8,000 33 U.S.C. 1608(a) International Navigation Rules; Operator 8,000 33 U.S.C. 1608(b) International Navigation Rules; Vessel 8,000 33 U.S.C. 1908(b)(1) Pollution from Ships; General 40,000 33 U.S.C... (Judicial Assessment subsequent offense) 100,000 46 U.S.C. App 1505(a)(2) Safe Containers for International...

7 CFR Appendix to Subpart F of... - Unknown Title

Code of Federal Regulations, 2013 CFR

2013-01-01

... U.S.C. 1773. 15. Special Food Service Program for Children. 42 U.S.C. 1761. 16. Special Supplemental Food Program for Women, Infants, and Children. 42 U.S.C. 1786. 17. Cash grants to States for nutrition... development programs. 40 U.S.C. 204. 8. Advance of funds for cooperative research. 16 U.S.C. 581i-l. 9...

7 CFR Appendix to Subpart F of... - Unknown Title

Code of Federal Regulations, 2010 CFR

2010-01-01

... U.S.C. 1773. 15. Special Food Service Program for Children. 42 U.S.C. 1761. 16. Special Supplemental Food Program for Women, Infants, and Children. 42 U.S.C. 1786. 17. Cash grants to States for nutrition... development programs. 40 U.S.C. 204. 8. Advance of funds for cooperative research. 16 U.S.C. 581i-l. 9...

7 CFR Appendix to Subpart F of... - Unknown Title

Code of Federal Regulations, 2012 CFR

2012-01-01

... U.S.C. 1773. 15. Special Food Service Program for Children. 42 U.S.C. 1761. 16. Special Supplemental Food Program for Women, Infants, and Children. 42 U.S.C. 1786. 17. Cash grants to States for nutrition... development programs. 40 U.S.C. 204. 8. Advance of funds for cooperative research. 16 U.S.C. 581i-l. 9...

7 CFR Appendix to Subpart F of... - Unknown Title

Code of Federal Regulations, 2011 CFR

2011-01-01

... U.S.C. 1773. 15. Special Food Service Program for Children. 42 U.S.C. 1761. 16. Special Supplemental Food Program for Women, Infants, and Children. 42 U.S.C. 1786. 17. Cash grants to States for nutrition... development programs. 40 U.S.C. 204. 8. Advance of funds for cooperative research. 16 U.S.C. 581i-l. 9...

7 CFR Appendix to Subpart F of... - Unknown Title

Code of Federal Regulations, 2014 CFR

2014-01-01

... U.S.C. 1773. 15. Special Food Service Program for Children. 42 U.S.C. 1761. 16. Special Supplemental Food Program for Women, Infants, and Children. 42 U.S.C. 1786. 17. Cash grants to States for nutrition... development programs. 40 U.S.C. 204. 8. Advance of funds for cooperative research. 16 U.S.C. 581i-l. 9...

77 FR 48203 - Final Federal Agency Actions on Proposed Highway in North Carolina

Federal Register 2010, 2011, 2012, 2013, 2014

2012-08-13

... Wetlands Mitigation [23 U.S.C. 103(b)(6)(m), 133(b)(11)]; Flood Disaster Protection Act [42 U.S.C. 4001... Species Act [16 USC 1531-1544 and Section 1536], Marine Mammal Protection Act [16 U.S.C. 1361], Anadromous....]; Archeological Resources Protection Act of 1977 [16 U.S.C. 470(aa)-11]; Archeological and Historic Preservation...

77 FR 7946 - Regulatory Agenda

Federal Register 2010, 2011, 2012, 2013, 2014

2012-02-13

... for Infusion Pumps. 344 Requirements for the 0910-AG59 Testing and Reporting of Tobacco Product... Infusion Pumps Legal Authority: 21 U.S.C. 351; 21 U.S.C. 360; 21 U.S.C. 360c; 21 U.S.C. 360e; 21 U.S.C... infusion pumps. An analysis of the reports reveals that a majority of the recalls and failures were caused...

50 CFR 12.6 - Bonded release.

Code of Federal Regulations, 2014 CFR

2014-10-01

..., payment of the value as determined under § 12.12) in place of any property seized under the Endangered Species Act, 16 U.S.C. 1531 et seq.; Marine Mammal Protection Act, 16 U.S.C. 1361 et seq.; Lacey Act, 18 U.S.C. 43; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Airborne Hunting Act, 16 U.S.C. 742j...

50 CFR 12.12 - Appraisement.

Code of Federal Regulations, 2011 CFR

2011-10-01

... Protection Act, 16 U.S.C. 1361 et seq., and the value of any property seized under the Endangered Species Act, 16 U.S.C. 1531 et seq.; Eagle Protection Act, 16 U.S.C. 668 et seq.; Airborne Hunting Act, 16 U.S.C. 742j-1, et seq.; or the Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq. If the seized property may...

50 CFR 12.12 - Appraisement.

Code of Federal Regulations, 2010 CFR

2010-10-01

... Protection Act, 16 U.S.C. 1361 et seq., and the value of any property seized under the Endangered Species Act, 16 U.S.C. 1531 et seq.; Eagle Protection Act, 16 U.S.C. 668 et seq.; Airborne Hunting Act, 16 U.S.C. 742j-1, et seq.; or the Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq. If the seized property may...

50 CFR 12.2 - Scope of regulations.

Code of Federal Regulations, 2012 CFR

2012-10-01

... Act, 16 U.S.C. 1361 et seq.; (h) The Endangered Species Act, 16 U.S.C. 1531 et seq.; and (i) The Lacey... the following laws: (a) The Eagle Protection Act, 16 U.S.C. 668 et seq.; (b) The National Wildlife Refuge System Administration Act, 16 U.S.C. 668dd et seq.; (c) The Migratory Bird Treaty Act, 16 U.S.C...

50 CFR 12.2 - Scope of regulations.

Code of Federal Regulations, 2014 CFR

2014-10-01

... Act, 16 U.S.C. 1361 et seq.; (h) The Endangered Species Act, 16 U.S.C. 1531 et seq.; and (i) The Lacey... the following laws: (a) The Eagle Protection Act, 16 U.S.C. 668 et seq.; (b) The National Wildlife Refuge System Administration Act, 16 U.S.C. 668dd et seq.; (c) The Migratory Bird Treaty Act, 16 U.S.C...

50 CFR 12.12 - Appraisement.

Code of Federal Regulations, 2014 CFR

2014-10-01

... Protection Act, 16 U.S.C. 1361 et seq., and the value of any property seized under the Endangered Species Act, 16 U.S.C. 1531 et seq.; Eagle Protection Act, 16 U.S.C. 668 et seq.; Airborne Hunting Act, 16 U.S.C. 742j-1, et seq.; or the Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq. If the seized property may...

43 CFR 8340.0-3 - Authority.

Code of Federal Regulations, 2011 CFR

2011-10-01

... of this part are issued under the Federal Land Policy and Management Act of 1976 (43 U.S.C. 1701 et seq.); the Taylor Grazing Act (43 U.S.C. 315a); the Endangered Species Act (16 U.S.C. 1531 et seq.); the Wild and Scenic Rivers Act (16 U.S.C. 1281c); the Act of September 15, 1960, as amended (16 U.S.C...

50 CFR 12.6 - Bonded release.

Code of Federal Regulations, 2010 CFR

2010-10-01

..., payment of the value as determined under § 12.12) in place of any property seized under the Endangered Species Act, 16 U.S.C. 1531 et seq.; Marine Mammal Protection Act, 16 U.S.C. 1361 et seq.; Lacey Act, 18 U.S.C. 43; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Airborne Hunting Act, 16 U.S.C. 742j...

43 CFR 8340.0-3 - Authority.

Code of Federal Regulations, 2012 CFR

2012-10-01

... of this part are issued under the Federal Land Policy and Management Act of 1976 (43 U.S.C. 1701 et seq.); the Taylor Grazing Act (43 U.S.C. 315a); the Endangered Species Act (16 U.S.C. 1531 et seq.); the Wild and Scenic Rivers Act (16 U.S.C. 1281c); the Act of September 15, 1960, as amended (16 U.S.C...

50 CFR 12.6 - Bonded release.

Code of Federal Regulations, 2011 CFR

2011-10-01

..., payment of the value as determined under § 12.12) in place of any property seized under the Endangered Species Act, 16 U.S.C. 1531 et seq.; Marine Mammal Protection Act, 16 U.S.C. 1361 et seq.; Lacey Act, 18 U.S.C. 43; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Airborne Hunting Act, 16 U.S.C. 742j...

50 CFR 12.12 - Appraisement.

Code of Federal Regulations, 2012 CFR

2012-10-01

... Protection Act, 16 U.S.C. 1361 et seq., and the value of any property seized under the Endangered Species Act, 16 U.S.C. 1531 et seq.; Eagle Protection Act, 16 U.S.C. 668 et seq.; Airborne Hunting Act, 16 U.S.C. 742j-1, et seq.; or the Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq. If the seized property may...

50 CFR 12.12 - Appraisement.

Code of Federal Regulations, 2013 CFR

2013-10-01

... Protection Act, 16 U.S.C. 1361 et seq., and the value of any property seized under the Endangered Species Act, 16 U.S.C. 1531 et seq.; Eagle Protection Act, 16 U.S.C. 668 et seq.; Airborne Hunting Act, 16 U.S.C. 742j-1, et seq.; or the Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq. If the seized property may...

43 CFR 8340.0-3 - Authority.

Code of Federal Regulations, 2014 CFR

2014-10-01

... of this part are issued under the Federal Land Policy and Management Act of 1976 (43 U.S.C. 1701 et seq.); the Taylor Grazing Act (43 U.S.C. 315a); the Endangered Species Act (16 U.S.C. 1531 et seq.); the Wild and Scenic Rivers Act (16 U.S.C. 1281c); the Act of September 15, 1960, as amended (16 U.S.C...

50 CFR 12.2 - Scope of regulations.

Code of Federal Regulations, 2011 CFR

2011-10-01

... Act, 16 U.S.C. 1361 et seq.; (h) The Endangered Species Act, 16 U.S.C. 1531 et seq.; and (i) The Lacey... the following laws: (a) The Eagle Protection Act, 16 U.S.C. 668 et seq.; (b) The National Wildlife Refuge System Administration Act, 16 U.S.C. 668dd et seq.; (c) The Migratory Bird Treaty Act, 16 U.S.C...

50 CFR 12.6 - Bonded release.

Code of Federal Regulations, 2013 CFR

2013-10-01

..., payment of the value as determined under § 12.12) in place of any property seized under the Endangered Species Act, 16 U.S.C. 1531 et seq.; Marine Mammal Protection Act, 16 U.S.C. 1361 et seq.; Lacey Act, 18 U.S.C. 43; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Airborne Hunting Act, 16 U.S.C. 742j...

43 CFR 8340.0-3 - Authority.

Code of Federal Regulations, 2013 CFR

2013-10-01

... of this part are issued under the Federal Land Policy and Management Act of 1976 (43 U.S.C. 1701 et seq.); the Taylor Grazing Act (43 U.S.C. 315a); the Endangered Species Act (16 U.S.C. 1531 et seq.); the Wild and Scenic Rivers Act (16 U.S.C. 1281c); the Act of September 15, 1960, as amended (16 U.S.C...

50 CFR 12.2 - Scope of regulations.

Code of Federal Regulations, 2013 CFR

2013-10-01

... Act, 16 U.S.C. 1361 et seq.; (h) The Endangered Species Act, 16 U.S.C. 1531 et seq.; and (i) The Lacey... the following laws: (a) The Eagle Protection Act, 16 U.S.C. 668 et seq.; (b) The National Wildlife Refuge System Administration Act, 16 U.S.C. 668dd et seq.; (c) The Migratory Bird Treaty Act, 16 U.S.C...

50 CFR 12.6 - Bonded release.

Code of Federal Regulations, 2012 CFR

2012-10-01

..., payment of the value as determined under § 12.12) in place of any property seized under the Endangered Species Act, 16 U.S.C. 1531 et seq.; Marine Mammal Protection Act, 16 U.S.C. 1361 et seq.; Lacey Act, 18 U.S.C. 43; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Airborne Hunting Act, 16 U.S.C. 742j...

50 CFR 12.2 - Scope of regulations.

Code of Federal Regulations, 2010 CFR

2010-10-01

... Act, 16 U.S.C. 1361 et seq.; (h) The Endangered Species Act, 16 U.S.C. 1531 et seq.; and (i) The Lacey... the following laws: (a) The Eagle Protection Act, 16 U.S.C. 668 et seq.; (b) The National Wildlife Refuge System Administration Act, 16 U.S.C. 668dd et seq.; (c) The Migratory Bird Treaty Act, 16 U.S.C...

23 CFR 420.103 - How does the FHWA define the terms used in this part?

Code of Federal Regulations, 2010 CFR

2010-04-01

... provisions of 23 U.S.C. 134); (3) National highway system (NHS) funds authorized under 23 U.S.C. 104(b)(1... and establishment of management systems under 23 U.S.C. 303; (4) Surface transportation program (STP... management systems under 23 U.S.C. 303; and (5) Minimum guarantee (MG) funds authorized under 23 U.S.C. 505...

23 CFR Appendix A to Part 773 - FHWA Environmental Responsibilities That May Be Assigned Under Section 6005

Code of Federal Regulations, 2012 CFR

2012-04-01

... Section 1536 Marine Mammal Protection Act, 16 U.S.C. 1361 Anadromous Fish Conservation Act, 16 U.S.C. 757... 1966, as amended, 16 U.S.C. 470(f) et seq. Archeological Resources Protection Act of 1977, 16 U.S.C... Protection and Repatriation Act (NAGPRA), 25 U.S.C. 3001-3013 Social and Economic Impacts American Indian...

23 CFR Appendix A to Part 773 - FHWA Environmental Responsibilities That May Be Assigned Under Section 6005

Code of Federal Regulations, 2013 CFR

2013-04-01

... Section 1536 Marine Mammal Protection Act, 16 U.S.C. 1361 Anadromous Fish Conservation Act, 16 U.S.C. 757... 1966, as amended, 16 U.S.C. 470(f) et seq. Archeological Resources Protection Act of 1977, 16 U.S.C... Protection and Repatriation Act (NAGPRA), 25 U.S.C. 3001-3013 Social and Economic Impacts American Indian...

23 CFR Appendix A to Part 773 - FHWA Environmental Responsibilities That May Be Assigned Under Section 6005

Code of Federal Regulations, 2014 CFR

2014-04-01

... Section 1536 Marine Mammal Protection Act, 16 U.S.C. 1361 Anadromous Fish Conservation Act, 16 U.S.C. 757... 1966, as amended, 16 U.S.C. 470(f) et seq. Archeological Resources Protection Act of 1977, 16 U.S.C... Protection and Repatriation Act (NAGPRA), 25 U.S.C. 3001-3013 Social and Economic Impacts American Indian...

23 CFR Appendix A to Part 773 - FHWA Environmental Responsibilities That May Be Assigned Under Section 6005

Code of Federal Regulations, 2010 CFR

2010-04-01

... Section 1536 Marine Mammal Protection Act, 16 U.S.C. 1361 Anadromous Fish Conservation Act, 16 U.S.C. 757... 1966, as amended, 16 U.S.C. 470(f) et seq. Archeological Resources Protection Act of 1977, 16 U.S.C... Protection and Repatriation Act (NAGPRA), 25 U.S.C. 3001-3013 Social and Economic Impacts American Indian...

23 CFR Appendix A to Part 773 - FHWA Environmental Responsibilities That May Be Assigned Under Section 6005

Code of Federal Regulations, 2011 CFR

2011-04-01

... Section 1536 Marine Mammal Protection Act, 16 U.S.C. 1361 Anadromous Fish Conservation Act, 16 U.S.C. 757... 1966, as amended, 16 U.S.C. 470(f) et seq. Archeological Resources Protection Act of 1977, 16 U.S.C... Protection and Repatriation Act (NAGPRA), 25 U.S.C. 3001-3013 Social and Economic Impacts American Indian...

45 CFR 97.12 - Which grants may be consolidated?

Code of Federal Regulations, 2011 CFR

2011-10-01

... in § 97.12(a)(1). (4) Social Services, 42 U.S.C. 1397-1397f. (5) Community Services, 42 U.S.C. 9901-9912. (6) Low-Income Home Energy Assistance, 42 U.S.C. 8621-8629. (7) Community Youth Activity, 42 U.S... Violence Prevention and Services, 42 U.S.C. 10401, et seq. (9) Children's Justice Act, 42 U.S.C. 5101, et...

50 CFR 12.25 - Transfers in settlement of civil penalty claims.

Code of Federal Regulations, 2010 CFR

2010-10-01

... may be liable for civil penalty under the Endangered Species Act, 16 U.S.C. 1531 et seq.; Lacey Act, 18 U.S.C. 43; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Eagle Protection Act, 16 U.S.C. 668 et seq.; or Marine Mammal Protection Act, 16 U.S.C. 1361 et seq., may be given an opportunity to...

25 CFR Appendix A to Subpart D - Cultural Resource and Environmental Requirements for the IRR Program

Code of Federal Regulations, 2011 CFR

2011-04-01

... limited to: 1. 16 U.S.C. 1531, Endangered Species Act. 2. 16 U.S.C. 4601, Land and Water Conservation Fund Act (Section 6(f)). 3. 16 U.S.C. 661-667d, Fish and Wildlife Coordination Act. 4. 23 U.S.C. 138.... 10. 7 U.S.C. 4201, Farmland Protection Policy Act. 11. 50 CFR part 402, Endangered Species Act...

50 CFR 12.25 - Transfers in settlement of civil penalty claims.

Code of Federal Regulations, 2011 CFR

2011-10-01

... may be liable for civil penalty under the Endangered Species Act, 16 U.S.C. 1531 et seq.; Lacey Act, 18 U.S.C. 43; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Eagle Protection Act, 16 U.S.C. 668 et seq.; or Marine Mammal Protection Act, 16 U.S.C. 1361 et seq., may be given an opportunity to...

25 CFR Appendix A to Subpart D - Cultural Resource and Environmental Requirements for the IRR Program

Code of Federal Regulations, 2012 CFR

2012-04-01

... limited to: 1. 16 U.S.C. 1531, Endangered Species Act. 2. 16 U.S.C. 4601, Land and Water Conservation Fund Act (Section 6(f)). 3. 16 U.S.C. 661-667d, Fish and Wildlife Coordination Act. 4. 23 U.S.C. 138.... 10. 7 U.S.C. 4201, Farmland Protection Policy Act. 11. 50 CFR part 402, Endangered Species Act...

50 CFR 12.25 - Transfers in settlement of civil penalty claims.

Code of Federal Regulations, 2014 CFR

2014-10-01

... may be liable for civil penalty under the Endangered Species Act, 16 U.S.C. 1531 et seq.; Lacey Act, 18 U.S.C. 43; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Eagle Protection Act, 16 U.S.C. 668 et seq.; or Marine Mammal Protection Act, 16 U.S.C. 1361 et seq., may be given an opportunity to...

50 CFR 12.22 - Civil actions to obtain forfeiture.

Code of Federal Regulations, 2012 CFR

2012-10-01

... Endangered Species Act, 16 U.S.C. 1531 et seq. Before any such action is filed against property subject to... property subject to forfeiture under the Airborne Hunting Act, 16 U.S.C. 742j-1; Lacey Act, 18 U.S.C. 43-44; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Black Bass Act, 16 U.S.C. 851 et seq.; Marine...

50 CFR 12.22 - Civil actions to obtain forfeiture.

Code of Federal Regulations, 2014 CFR

2014-10-01

... Endangered Species Act, 16 U.S.C. 1531 et seq. Before any such action is filed against property subject to... property subject to forfeiture under the Airborne Hunting Act, 16 U.S.C. 742j-1; Lacey Act, 18 U.S.C. 43-44; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Black Bass Act, 16 U.S.C. 851 et seq.; Marine...

25 CFR Appendix A to Subpart D - Cultural Resource and Environmental Requirements for the IRR Program

Code of Federal Regulations, 2013 CFR

2013-04-01

... limited to: 1. 16 U.S.C. 1531, Endangered Species Act. 2. 16 U.S.C. 4601, Land and Water Conservation Fund Act (Section 6(f)). 3. 16 U.S.C. 661-667d, Fish and Wildlife Coordination Act. 4. 23 U.S.C. 138.... 10. 7 U.S.C. 4201, Farmland Protection Policy Act. 11. 50 CFR part 402, Endangered Species Act...

50 CFR 12.25 - Transfers in settlement of civil penalty claims.

Code of Federal Regulations, 2012 CFR

2012-10-01

... may be liable for civil penalty under the Endangered Species Act, 16 U.S.C. 1531 et seq.; Lacey Act, 18 U.S.C. 43; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Eagle Protection Act, 16 U.S.C. 668 et seq.; or Marine Mammal Protection Act, 16 U.S.C. 1361 et seq., may be given an opportunity to...

50 CFR 12.22 - Civil actions to obtain forfeiture.

Code of Federal Regulations, 2010 CFR

2010-10-01

... Endangered Species Act, 16 U.S.C. 1531 et seq. Before any such action is filed against property subject to... property subject to forfeiture under the Airborne Hunting Act, 16 U.S.C. 742j-1; Lacey Act, 18 U.S.C. 43-44; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Black Bass Act, 16 U.S.C. 851 et seq.; Marine...

50 CFR 12.22 - Civil actions to obtain forfeiture.

Code of Federal Regulations, 2013 CFR

2013-10-01

... Endangered Species Act, 16 U.S.C. 1531 et seq. Before any such action is filed against property subject to... property subject to forfeiture under the Airborne Hunting Act, 16 U.S.C. 742j-1; Lacey Act, 18 U.S.C. 43-44; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Black Bass Act, 16 U.S.C. 851 et seq.; Marine...

50 CFR 12.22 - Civil actions to obtain forfeiture.

Code of Federal Regulations, 2011 CFR

2011-10-01

... Endangered Species Act, 16 U.S.C. 1531 et seq. Before any such action is filed against property subject to... property subject to forfeiture under the Airborne Hunting Act, 16 U.S.C. 742j-1; Lacey Act, 18 U.S.C. 43-44; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Black Bass Act, 16 U.S.C. 851 et seq.; Marine...

25 CFR Appendix A to Subpart D - Cultural Resource and Environmental Requirements for the IRR Program

Code of Federal Regulations, 2014 CFR

2014-04-01

... limited to: 1. 16 U.S.C. 1531, Endangered Species Act. 2. 16 U.S.C. 4601, Land and Water Conservation Fund Act (Section 6(f)). 3. 16 U.S.C. 661-667d, Fish and Wildlife Coordination Act. 4. 23 U.S.C. 138.... 10. 7 U.S.C. 4201, Farmland Protection Policy Act. 11. 50 CFR part 402, Endangered Species Act...

50 CFR 12.25 - Transfers in settlement of civil penalty claims.

Code of Federal Regulations, 2013 CFR

2013-10-01

... may be liable for civil penalty under the Endangered Species Act, 16 U.S.C. 1531 et seq.; Lacey Act, 18 U.S.C. 43; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Eagle Protection Act, 16 U.S.C. 668 et seq.; or Marine Mammal Protection Act, 16 U.S.C. 1361 et seq., may be given an opportunity to...

15 CFR 904.1 - Purpose and scope.

Code of Federal Regulations, 2012 CFR

2012-01-01

... Bass Conservation Act, 16 U.S.C. 1851 note; (9) Atlantic Tunas Convention Act of 1975, 16 U.S.C. 971... Seal Act Amendments of 1983, 16 U.S.C. 1151-1175; (18) High Seas Fishing Compliance Act, 16 U.S.C. 5506...

15 CFR 904.1 - Purpose and scope.

Code of Federal Regulations, 2013 CFR

2013-01-01

... Bass Conservation Act, 16 U.S.C. 1851 note; (9) Atlantic Tunas Convention Act of 1975, 16 U.S.C. 971... Seal Act Amendments of 1983, 16 U.S.C. 1151-1175; (18) High Seas Fishing Compliance Act, 16 U.S.C. 5506...

Usual source of care and the quality of primary care: a survey of patients in Guangdong province, China.

PubMed

Du, Zhicheng; Liao, Yu; Chen, Chien-Chou; Hao, Yuantao; Hu, Ruwei

2015-07-31

Usual source of care (USC) refers to the provider or place a patient consults when sick or in need of medical advice. No studies have been conducted in China to compare the quality of primary care provided with or without USC. The purpose of this study was to fill this gap in the literature by examining the quality of primary care provided between those having a USC and those without. Results of the study would provide implications for policymakers in terms of improving primary care performance in China, and help guide patients in their health care seeking behaviors. A cross-sectional survey with patients was conducted in Guangdong province of China, using the Chinese validated Primary Care Assessment Tool (PCAT). ANOVA was performed to compare the overall and ten domains of primary care quality for patients with and without USC. Multivariate analyses were used to assess the association between USC and quality of primary care attributes while controlling for sociodemographic and health care characteristics. The study added evidence that having a USC can provide higher quality of primary care to patients than those without a USC. Results of this study showed that the PCAT score associated with those having a USC was significantly higher than those not having a USC. Moreover, the study showed that having a usual provider of care was also independently and significantly associated with patients' satisfaction with care. This study added evidence that in China, patients with a USC reported higher quality of medical care experiences compared with those without a USC. The efforts to improve quality of care should include policies promoting USC.

VizieR Online Data Catalog: Boo-127 and Boo-980 high-resolution spectra (Frebel+, 2016)

NASA Astrophysics Data System (ADS)

Frebel, A.; Norris, J. E.; Gilmore, G.; Wyse, R. F. G.

2016-09-01

We observed Boo-980 and Boo-127 stars with the MIKE spectrograph on the Magellan-Clay telescope in 2010 March and 2011 March. Details of the MIKE observations and photometry taken from Norris et al. (2008ApJ...689L.113N) are given in Table 1. MIKE spectra have nearly full optical wavelength coverage over the range ~3500-9000Å (R~22000 in the red and ~28000 in the blue wavelength regime). (2 data files).

The Response of Frozen Soils to Vibratory Loads

DTIC Science & Technology

1975-06-01

Construction. i | The report was technically reviewed by Dr. Y . Nakano of USA CRREL, and A.F. Müller of the Office of Chief of Engineers. Their suggestions...B.I.S. Helme, Jr., t M.J. Dabney III, F. Berrego, R.N. Lachenmaier and D.J. Coombes. Dr. T.M. Lee, Dr. D.M. Norris, Jr. and Dr. Y . Nakano gave... y /g stress static confining pressure, (a, + 2a ^/3 axial (vertical) static pressure lateral static pressure dynamic stress (peak) phase shift

Some Results in the Theory of Subset Selection Procedures.

DTIC Science & Technology

1980-08-01

kio( hho (’,r (1 ,’.P ), , u rc (1 , 9A, 1q64, 1967) and Bechhofer, Fiefor and obol (196,). Ii , n aid Gupta (107?), Huanq (197:’ , Glp ta an I llcd (1...lor the ,pec i.i I cosa n ( If F is; norr;.-i Ill tr’ ht)LJI o ~ is fyinrq the Equa ti on I. ’. .5 0’’eeo I " I’ :’O( hho f pr (1 19114 t w ~P>K 1 ehwl

Henry Norris Russell's Toronto Lectures

NASA Astrophysics Data System (ADS)

Devorkin, D. H.

1996-12-01

In February 1924, at the invitation of C. A. Chant, Russell presented a set of 14 public lectures on the state of astronomy and astrophysics. Designed to be inspirational, they also reveal Russell's contemporary views on the state of astrophysics as well as his sense of proper practice in astronomy. During his visit, Russell was interviewed by local reporters who asked his opinion about building a large observatory, one of Chant's major projects. What Russell had to say about such ventures did not please Chant one bit.

Enhancing the Efficacy of Dendritic Cell Vaccines by Tissue Conditioning

DTIC Science & Technology

2009-03-01

oponents of evidence based medicine in urology. Several peer-reviewed publications document this positive career de velopment.2-11 He ha s r ecently di...be invited as faculty of the prestigious workshops on how to teach evidence based medicine at Duke, M cMaster a nd Oxford U niversity. H e c ontinues... Based Medicine Review Articles. Journal of Urology, 178: 1149, 2007 3. Scales, C. D., Jr., Norris, R. D., Keitz, S. A., Peterson, B. L., Preminger

Molecular genetics of inherited eye disorders.

PubMed

MacDonald, I M; Sasi, R

1994-10-01

In the past 10 y, there have been considerable advances in the mapping, isolation, and characterization of many genes for important ocular conditions: retinitis pigmentosa, Norrie disease, Waardenburg syndrome, choroideremia, aniridia, retinoblastoma, and others. The candidate gene approach has now supplemented classical linkage studies and positional cloning in the investigation of ocular disorders. Developmentally expressed genes and animal models have provided insights as to the etiology of other disorders. With this knowledge at hand, genetic counselling for heritable eye diseases has been greatly improved.

78 FR 77198 - Notice of Final Federal Agency Actions on Proposed Highway in California

Federal Register 2010, 2011, 2012, 2013, 2014

2013-12-20

...: 23 U.S.C. 139(l)(1), as amended by the Moving Ahead for Progress in the 21st Century Act (MAP-21...: Endangered Species Act (16 U.S.C. 1531-1544 and Section 1536), Marine Mammal Protection Act [16 U.S.C. 1361], Fish and Wildlife Coordination Act (16 U.S.C. 661-667(d)), Migratory Bird Treaty Act (16 U.S.C. 703-712...

12 CFR 109.103 - Civil money penalties.

Code of Federal Regulations, 2012 CFR

2012-01-01

.... 1467a(r)(1) Late/Inaccurate Reports—1st Tier 2,200 12 U.S.C. 1467a(r)(2) Late/Inaccurate Reports—2nd Tier 32,500 12 U.S.C. 1467a(r)(3) Late/Inaccurate Reports—3rd Tier 1,375,000 12 U.S.C. 1817(j)(16)(A...,375,000 12 U.S.C. 1820(k)(6)(A)(ii) Violation of Post Employment Restrictions 275,000 12 U.S.C. 1884...

47 CFR 1.736 - Complaints filed pursuant to 47 U.S.C. 271(d)(6)(B).

Code of Federal Regulations, 2010 CFR

2010-10-01

... Complaints filed pursuant to 47 U.S.C. 271(d)(6)(B). (a) Where a complaint is filed pursuant to 47 U.S.C. 271... deadline contained in 47 U.S.C. 271(d)(6)(B) in the following manner: (1) The complainant shall so indicate... filed pursuant to 47 U.S.C. 271(d)(6)(B) will not be entertained by the Commission staff subsequent to...

47 CFR 1.736 - Complaints filed pursuant to 47 U.S.C. 271(d)(6)(B).

Code of Federal Regulations, 2014 CFR

2014-10-01

... Complaints filed pursuant to 47 U.S.C. 271(d)(6)(B). (a) Where a complaint is filed pursuant to 47 U.S.C. 271... deadline contained in 47 U.S.C. 271(d)(6)(B) in the following manner: (1) The complainant shall so indicate... filed pursuant to 47 U.S.C. 271(d)(6)(B) will not be entertained by the Commission staff subsequent to...

47 CFR 1.736 - Complaints filed pursuant to 47 U.S.C. 271(d)(6)(B).

Code of Federal Regulations, 2011 CFR

2011-10-01

... Complaints filed pursuant to 47 U.S.C. 271(d)(6)(B). (a) Where a complaint is filed pursuant to 47 U.S.C. 271... deadline contained in 47 U.S.C. 271(d)(6)(B) in the following manner: (1) The complainant shall so indicate... filed pursuant to 47 U.S.C. 271(d)(6)(B) will not be entertained by the Commission staff subsequent to...

47 CFR 1.736 - Complaints filed pursuant to 47 U.S.C. 271(d)(6)(B).

Code of Federal Regulations, 2012 CFR

2012-10-01

... Complaints filed pursuant to 47 U.S.C. 271(d)(6)(B). (a) Where a complaint is filed pursuant to 47 U.S.C. 271... deadline contained in 47 U.S.C. 271(d)(6)(B) in the following manner: (1) The complainant shall so indicate... filed pursuant to 47 U.S.C. 271(d)(6)(B) will not be entertained by the Commission staff subsequent to...

47 CFR 1.736 - Complaints filed pursuant to 47 U.S.C. 271(d)(6)(B).

Code of Federal Regulations, 2013 CFR

2013-10-01

... Complaints filed pursuant to 47 U.S.C. 271(d)(6)(B). (a) Where a complaint is filed pursuant to 47 U.S.C. 271... deadline contained in 47 U.S.C. 271(d)(6)(B) in the following manner: (1) The complainant shall so indicate... filed pursuant to 47 U.S.C. 271(d)(6)(B) will not be entertained by the Commission staff subsequent to...

7 CFR 400.130 - Notice requirements before offset.

Code of Federal Regulations, 2010 CFR

2010-01-01

..., and 1002, or any other applicable statutory authority; (l) Any other rights or remedies available to... (5 U.S.C. 5584, 10 U.S.C. 2774, 32 U.S.C 716, or 5 U.S.C 8346(b)), or may request waiver in the case...

76 FR 46807 - Agency Information Collection Activities: Announcement of Board Approval Under Delegated...

Federal Register 2010, 2011, 2012, 2013, 2014

2011-08-03

... collections are mandatory. FR 2030 and FR 2030a: (12 U.S.C. 222, 282, 248(a) and 321). FR 2056: (12 U.S.C. 287, 248(a) and (i)). FR 2086: (12 U.S.C. 287, 248(a) and (i)). FR 2086a: (12 U.S.C. 321, 287, 248(a)). FR... to obtain a benefit (12 U.S.C. 338a, and 12 CFR 208.22). Individual respondent data generally are not...

12 CFR 509.103 - Civil money penalties.

Code of Federal Regulations, 2014 CFR

2014-01-01

... Company Act Violation 32,500 12 U.S.C. 1467a(i)(3) Holding Company Act Violation 32,500 12 U.S.C. 1467a(r)(1) Late/Inaccurate Reports—1st Tier 2,200 12 U.S.C. 1467a(r)(2) Late/Inaccurate Reports—2nd Tier 32,500 12 U.S.C. 1467a(r)(3) Late/Inaccurate Reports—3rd Tier 1,375,000 12 U.S.C. 1817(j)(16)(A) Change...

12 CFR 509.103 - Civil money penalties.

Code of Federal Regulations, 2012 CFR

2012-01-01

... Company Act Violation 32,500 12 U.S.C. 1467a(i)(3) Holding Company Act Violation 32,500 12 U.S.C. 1467a(r)(1) Late/Inaccurate Reports—1st Tier 2,200 12 U.S.C. 1467a(r)(2) Late/Inaccurate Reports—2nd Tier 32,500 12 U.S.C. 1467a(r)(3) Late/Inaccurate Reports—3rd Tier 1,375,000 12 U.S.C. 1817(j)(16)(A) Change...

12 CFR 509.103 - Civil money penalties.

Code of Federal Regulations, 2013 CFR

2013-01-01

... Company Act Violation 32,500 12 U.S.C. 1467a(i)(3) Holding Company Act Violation 32,500 12 U.S.C. 1467a(r)(1) Late/Inaccurate Reports—1st Tier 2,200 12 U.S.C. 1467a(r)(2) Late/Inaccurate Reports—2nd Tier 32,500 12 U.S.C. 1467a(r)(3) Late/Inaccurate Reports—3rd Tier 1,375,000 12 U.S.C. 1817(j)(16)(A) Change...

12 CFR 509.103 - Civil money penalties.

Code of Federal Regulations, 2011 CFR

2011-01-01

... Company Act Violation 32,500 12 U.S.C. 1467a(i)(3) Holding Company Act Violation 32,500 12 U.S.C. 1467a(r)(1) Late/Inaccurate Reports—1st Tier 2,200 12 U.S.C. 1467a(r)(2) Late/Inaccurate Reports—2nd Tier 32,500 12 U.S.C. 1467a(r)(3) Late/Inaccurate Reports—3rd Tier 1,375,000 12 U.S.C. 1817(j)(16)(A) Change...

78 FR 4833 - Order Denying Export Privileges

Federal Register 2010, 2011, 2012, 2013, 2014

2013-01-23

... violating the International Emergency Economic Powers Act (50 U.S.C. 1701, et seq. (2000 & Supp. IV 2010... order issued under the International Emergency Economic Powers Act (50 U.S.C. 1701-1706); 18 U.S.C. 793... effect under the International Emergency Economic Powers Act (50 U.S.C. 1701, et seq. (2000 & Supp. IV...

Factors associated with having a usual source of care in an ethnically diverse sample of Asian American adults.

PubMed

Chang, Eva; Chan, Kitty S; Han, Hae-Ra

2014-09-01

Despite significant population increases, how Asian Americans ethnic subgroups vary in having a usual source of care (USC) is poorly understood. To examine how having a USC varies among Asian American ethnic subgroups (Chinese, Filipinos, Japanese, Koreans, Vietnamese, and South Asians), and the potential factors influencing variation in having a USC. Data were from 2005 and 2009 California Health Interview Survey. Logistic regressions and pair-wise comparisons were used to compare odds of having a USC among Asian ethnic adults (18-64 y) and to examine ethnicity-specific associations with immigration-related factors (English proficiency, length of residence, and living in an ethnically concordant neighborhood) and key enabling (employment, income, insurance), predisposing (education), and need (health status) factors. Models also adjusted for other sociodemographic factors. Significant differences in the magnitude of the variation and factors influencing having a USC were found across Asian subgroups. Korean and Japanese adults had 52%-69% lower adjusted odds of having a USC compared with Chinese. Among all Asian subgroups, uninsured adults had 85%-94% lower adjusted odds of having a USC. Patterns of associations with USC and key factors varied by specific Asian subgroup. Patterns of associations for USC varied by Asian subgroup, although uninsurance persisted significantly across all subgroups. Persistent variation and heterogenous associations suggest that targeted, ethnicity-specific policies and outreach are needed to improve having a USC for Asian American ethnic adults.

Inhibition of gamma-secretase activity impedes uterine serous carcinoma growth in a human xenograft model.

PubMed

Groeneweg, Jolijn W; Hall, Tracilyn R; Zhang, Ling; Kim, Minji; Byron, Virginia F; Tambouret, Rosemary; Sathayanrayanan, Sriram; Foster, Rosemary; Rueda, Bo R; Growdon, Whitfield B

2014-06-01

Uterine serous carcinoma (USC) represents an aggressive subtype of endometrial cancer. We sought to understand Notch pathway activity in USC and determine if pathway inhibition has anti-tumor activity. Patient USC tissue blocks were obtained and used to correlate clinical outcomes with Notch1 expression. Three established USC cell lines were treated with gamma-secretase inhibitor (GSI) in vitro. Mice harboring cell line derived or patient derived USC xenografts (PDXs) were treated with vehicle, GSI, paclitaxel and carboplatin (P/C), or combination GSI and P/C. Levels of cleaved Notch1 protein and Hes1 mRNA were determined in GSI treated samples. Statistical analysis was performed using the Wilcoxon rank sum and Kaplan-Meier methods. High nuclear Notch1 protein expression was observed in 58% of USC samples with no correlation with overall survival. GSI induced dose-dependent reductions in cell number and decreased levels of cleaved Notch1 protein and Hes1 mRNA in vitro. Treatment of mice with GSI led to decreased Hes1 mRNA expression in USC xenografts. In addition, GSI impeded tumor growth of cell line xenografts as well as UT1 USC PDXs. When GSI and P/C were combined, synergistic anti-tumor activity was observed in UT1 xenografts. Notch1 is expressed in a large subset of USC. GSI-mediated Notch pathway inhibition led to both reduced cell numbers in vitro and decreased tumor growth of USC some xenograft models. When combined with conventional chemotherapy, GSI augmented anti-tumor activity in one USC PDX line suggesting that targeting of the Notch signaling pathway is a potential therapeutic strategy for future investigation. Copyright © 2014 Elsevier Inc. All rights reserved.

Dual CCNE1/PIK3CA targeting is synergistic in CCNE1-amplified/PIK3CA-mutated uterine serous carcinomas in vitro and in vivo

PubMed Central

Cocco, Emiliano; Lopez, Salvatore; Black, Jonathan; Bellone, Stefania; Bonazzoli, Elena; Predolini, Federica; Ferrari, Francesca; Schwab, Carlton L; Menderes, Gulden; Zammataro, Luca; Buza, Natalia; Hui, Pei; Wong, Serena; Zhao, Siming; Bai, Yalai; Rimm, David L; Ratner, Elena; Litkouhi, Babak; Silasi, Dan-Arin; Azodi, Masoud; Schwartz, Peter E; Santin, Alessandro D

2016-01-01

Background: Clinical options for patients harbouring advanced/recurrent uterine serous carcinoma (USC), an aggressive variant of endometrial tumour, are very limited. Next-generation sequencing (NGS) data recently demonstrated that cyclin E1 (CCNE1) gene amplification and pik3ca driver mutations are common in USC and may therefore represent ideal therapeutic targets. Methods: Cyclin E1 expression was evaluated by immunohistochemistry (IHC) on 95 USCs. The efficacy of the cyclin-dependent kinase 2/9 inhibitor CYC065 was assessed on multiple primary USC cell lines with or without CCNE1 amplification. Cell-cycle analyses and knockdown experiments were performed to assess CYC065 targeting specificity. Finally, the in vitro and in vivo activity of CYC065, Taselisib (a PIK3CA inhibitor) and their combinations was tested on USC xenografts derived from CCNE1-amplified/pik3ca-mutated USCs. Results: We found that 89.5% of the USCs expressed CCNE1. CYC065 blocked cells in the G1 phase of the cell cycle and inhibited cell growth specifically in CCNE1-overexpressing USCs. Cyclin E1 knockdown conferred increased resistance to CYC065, whereas CYC065 treatment of xenografts derived from CCNE1-amplified USCs significantly reduced tumour growth. The combination of CYC065 and Taselisib demonstrated synergistic effect in vitro and was significantly more effective than single-agent treatment in decreasing tumour growth in xenografts of CCNE1-amplified/pik3ca-mutated USCs. Conclusions: Dual CCNE1/PIK3CA blockade may represent a novel therapeutic option for USC patients harbouring recurrent CCNE1-amplified/pi3kca-mutated tumours. PMID:27351214

Usual source of care as a health insurance substitute for U.S. adults with diabetes?

PubMed

DeVoe, Jennifer E; Tillotson, Carrie J; Wallace, Lorraine S

2009-06-01

The purpose of this study was to examine the effects of health insurance and/or a usual source of care (USC) on receipt of diabetic-specific services and health care barriers for U.S. adults with diabetes. Secondary analyses of data from 6,562 diabetic individuals aged >or=18 years from the nationally representative Medical Expenditure Panel Survey from 2002 to 2005 were performed. Outcome measures included receipt of seven diabetic services plus five barriers to care. More than 84% of diabetic individuals in the U.S. had full-year coverage and a USC; 2.3% had neither one. In multivariate analyses, the uninsured with no USC had one-fifth the odds of receiving A1C screening (odds ratio 0.23 [95% CI 0.14-0.38]) and one-tenth the odds of a blood pressure check (0.08 [0.05-0.15]), compared with insured diabetic individuals with a USC. Similarly, being uninsured without a USC was associated with 5.5 times the likelihood of unmet medical needs (5.51 [3.49-8.70]) and three times more delayed urgent care (3.13 [1.53-6.38]) compared with being insured with a USC. Among the two groups with either insurance or a USC, diabetic individuals with only a USC had rates of diabetes-specific care more similar to those of insured individuals with a USC. In contrast, those with only insurance were closer to the reference group with fewer barriers to care. Insured diabetic individuals with a USC were better off than those with only a USC, only insurance, or neither one. Policy reforms must target both the financing and the delivery systems to achieve increased receipt of diabetes services and decreased barriers to care.

The effect of the Family Health Strategy on usual source of care in Brazil: data from the 2013 National Health Survey (PNS 2013).

PubMed

Dourado, Inês; Medina, Maria Guadalupe; Aquino, Rosana

2016-11-17

A usual source of care (USC) has been conceptualized as having a health provider or place available for patients to consult when sick or in need of medical care. Having a USC is a means to achieve longitudinality of care with Primary Health Care (PHC) providers. Brazil has made enormous progress in PHC and thus provides an important opportunity to investigate USC in a middle-income country context. This study uses data from a nationally representative household survey, the 2013 National Health Survey (n = 62,986), to describe the prevalence of having a USC in Brazil and to investigate to what extent the Family Health Strategy (FHS) has contributed to USC prevalence. Analyses include descriptive, bivariate and multivariable Poisson regression. Show very high rates of people reporting any type of USC (74.4 %) and more than one third reporting PHC as their USC. Household enrolment in the FHS was positively associated with having any USC (PR:1.09; 95 % CI: 1.07-1.12) and a stronger association with having PHC as the regular source of care (PR:1.63;95 % CI:1.54-1.73). FHS enrolment was negatively associated with reporting emergency/urgent care facilities as one's USC (PR: 0.67; 95 % CI: 0.59-0.76). The association between the more consolidated FHS with having a USC was strongest in the poorest regions of the country (North, Northeast and Central-West). Having PHC as one's USC showed a positive dose-response relationship with the FHS in all regions, especially in the Central-West. Our results have important implications for the health care model in Brazil and in other countries, especially those seeking to base their national health systems more strongly on primary health care. The study suggests expanding primary health care can increase the establishment of a USC which can help assure better monitoring of chronic conditions and attention to patient needs.

Source and fate of inorganic solutes in the Gibbon River, Yellowstone National Park, Wyoming, USA. II. Trace element chemistry

USGS Publications Warehouse

McCleskey, R. Blaine; Nordstrom, D. Kirk; Susong, David D.; Ball, James W.; Taylor, Howard E.

2010-01-01

The Gibbon River in Yellowstone National Park receives inflows from several geothermal areas, and consequently the concentrations of many trace elements are elevated compared to rivers in non-geothermal watersheds. Water samples and discharge measurements were obtained from the Gibbon River and its major tributaries near Norris Geyser Basin under the low-flow conditions of September 2006 allowing for the identification of solute sources and their downstream fate. Norris Geyser Basin, and in particular Tantalus Creek, is the largest source of many trace elements (Al, As, B, Ba, Br, Cs, Hg, Li, Sb, Tl, W, and REEs) to the Gibbon River. The Chocolate Pots area is a major source of Fe and Mn, and the lower Gibbon River near Terrace Spring is the major source of Be and Mo. Some of the elevated trace elements are aquatic health concerns (As, Sb, and Hg) and knowing their fate is important. Most solutes in the Gibbon River, including As and Sb, behave conservatively or are minimally attenuated over 29 km of fluvial transport. Some small attenuation of Al, Fe, Hg, and REEs occurs but primarily there is a transformation from the dissolved state to suspended particles, with most of these elements still being transported to the Madison River. Dissolved Hg and REEs loads decrease where the particulate Fe increases, suggesting sorption onto suspended particulate material. Attenuation from the water column is substantial for Mn, with little formation of Mn as suspended particulates.

Phenotypic heterogeneity associated with a novel mutation (Gly112Glu) in the Norrie disease protein.

PubMed

Allen, R C; Russell, S R; Streb, L M; Alsheikheh, A; Stone, E M

2006-02-01

To determine the molecular pathology and clinical severity of two pedigrees with a history of early retinal detachment and peripheral retinal vascular abnormalities. Longitudinal cohort study. A longitudinal clinical study and DNA analysis was performed on 49 family members of two pedigrees. Nine individuals were found to be hemizygous for a mutation at codon 112 (Gly112Glu) of the Norrie disease protein (NDP) in one pedigree. Significant phenotypic heterogeneity was found. The proband presented with a unilateral subtotal retinal detachment at the age of 3 years, and subsequently developed a slowly progressive tractional retinal detachment involving the macula in the contralateral eye at the age of 4 years. One individual had only mild peripheral retinal pigmentary changes with normal vision at the age of 79 years. The remaining seven individuals had varying degrees of peripheral retinal vascular abnormalities and anterior segment findings. Seven affected members of a second pedigree affected by a previously reported mutation, Arg74Cys, also demonstrated wide ocular phenotypic variation. A novel mutation (Gly112Glu), which represents the most carboxy located, NDP mutation reported, results in significant phenotypic heterogeneity. These data support the contention that the spectrum of ocular disease severity associated with these NDP mutations is broad. Use of terms that characterize this entity by phenotypic appearance, such as familial exudative vitreoretinopathy, do not adequately communicate the potential spectrum of severity of this disorder to affected or carrier family members.

Prealternate molt in nuthatches

USGS Publications Warehouse

Banks, Richard C.

1978-01-01

Nuthatches of the genus Sitta constitute one of the passerine groups in which prealternate (prenuptial) molt is generally believed not to occur. Dwight (1900: 298) discussed this genus with the family Paridae and stated: "All the members of this family have only the annual moult. They are peculiar in assuming a juvenal plumage closely resembling the pale nuptial plumage which results from extreme fading of the winter dress." Witherby (in Witherby et al. 1938: 244) remarked that in S. europaea the summer plumage is attained by abrasion rather than by molt. Following Dwight's analysis, Bent (1948) recorded that the prenuptial molt did not occur in S. carolinensis or S. canadensis. Although Norris (1958: 246) found several females of S. pygmaea that were replacing feathers in the nuchal region in March and April, he regarded this as a regrowth of feathers lost in social interactions rather than as prealternate molt; the replacement of contour feathers "over nearly every part of the body" in early April by one female of that species was considered to be "an aberrant instance of prenuptial molt." Norris (op. cit.) stated categorically that "There is no prenuptial molt in Sitta pusilla." For most species in the genus, however, I have been unable to find any statement, even a negative one, concerning the occurrence of this molt. On the other hand, there is a partial prealternate molt in the seasonally dimorphic Tichodroma muraria, usually considered a member of the family Sittidae but in a different subfamily.

Pipeline corridors through wetlands -- Impacts on plant communities: Norris Brook Crossing Peabody, Massachusetts

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

Shem, L.M.; Van Dyke, G.D.; Zimmerman, R.E.

1994-12-01

The goal of the Gas Research Institute Wetland Corridors Program is to document impacts of existing pipelines on the wetlands they traverse. To accomplish this goal, 12 existing wetland crossings were surveyed. These sites varied in elapsed time since pipeline construction, wetland type, pipeline installation techniques, and right-of-way (ROW) management practices. This report presents the results of a survey conducted August 17--19, 1992, at the Norris Brook crossing in the town of Peabody, Essex County, Massachusetts. The pipeline at this site was installed during September and October 1990. A backhoe was used to install the pipeline. The pipe was assembledmore » on the adjacent upland and slid into the trench, after which the backhoe was used again to fill the trench and cover the pipeline. Within two years after pipeline construction, a dense vegetative community, composed predominantly of native perennial species, had become established on the ROW. Compared with adjacent natural areas undisturbed by pipeline installation, there was an increase in purple loosestrife and cattail within the ROW, while large woody species were excluded from the ROW. As a result of the ROW`s presence, habitat diversity, edge-type habitat, and species diversity increased within the site. Crooked-stem aster, Aster prenanthoides (a species on the Massasschusetts list of plants of special concern), occurred in low numbers in the adjacent natural areas and had reinvaded the ROW in low numbers.« less

Norrie gene product is necessary for regression of hyaloid vessels.

PubMed

Ohlmann, Anne V; Adamek, Edith; Ohlmann, Andreas; Lütjen-Drecoll, Elke

2004-07-01

To investigate the nature and origin of the vitreous membranes in mice with knock-out of the Norrie gene product (ND mice). Eighty-two eyes of ND mice of different age groups (postnatal day [P]0-13 months) and 95 age-matched wild-type control mice were investigated. In vitreoretinal wholemounts and in sagittal sections, vessels and free cells were visualized by labeling for lectin. In addition, staining with a marker for macrophages (F4/80) and collagen XVIII/endostatin known to be involved in regression of hyaloid vessels was performed for light and electron microscopic investigations. Endostatin expression was confirmed by Western blot analysis. Wild-type controls showed the typical pattern of hyaloid vessels, their regression and concomitantly retinal vasculogenesis and angiogenesis. Hyaloid vessels all stained for endostatin, whereas retinal vessels remained unstained. In ND mice, 1 to 5 days after birth, the hyaloid and retinal vasculatures were comparable to that in control mice. The hyaloid vessels also stained for endostatin. Numerous F4/80-positive cells were present adjacent to the vessels. With increasing age, only a few connecting branches of the hyaloid vessels regressed. Even in old mice most of the hyaloid vessels persisted. The vessels still stained for endostatin. Retinal angiogenesis was impaired. Retrolental membranes in ND mice consist of persistent hyaloid vessels, indicating that the ND gene product is important for the process of regression of these vessels. The ND gene product neither influences endostatin expression nor the presence of macrophages.

76 FR 65206 - Agency Information Collection Activities: Small Vessel Reporting System

Federal Register 2010, 2011, 2012, 2013, 2014

2011-10-20

... States. SVRS is authorized by 8 U.S.C. 1103, 8 U.S.C. 1225, 8 CFR 235.1, 19 U.S.C. 1433, 19 U.S.C. 1498... Review: Approval of a new collection. Affected Public: Individuals. Estimated Number of Respondents: 10... Filed 10-19-11; 8:45 am] BILLING CODE 9111-14-P ...

40 CFR 123.27 - Requirements for enforcement authority.

Code of Federal Regulations, 2013 CFR

2013-07-01

... established under § 123.34. (Clean Water Act (33 U.S.C. 1251 et seq.), Safe Drinking Water Act (42 U.S.C. 300f et seq.), Clean Air Act (42 U.S.C. 7401 et seq.), Resource Conservation and Recovery Act (42 U.S.C.... 123.27 Section 123.27 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) WATER...

40 CFR 123.27 - Requirements for enforcement authority.

Code of Federal Regulations, 2011 CFR

2011-07-01

... established under § 123.34. (Clean Water Act (33 U.S.C. 1251 et seq.), Safe Drinking Water Act (42 U.S.C. 300f et seq.), Clean Air Act (42 U.S.C. 7401 et seq.), Resource Conservation and Recovery Act (42 U.S.C.... 123.27 Section 123.27 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) WATER...

40 CFR 123.27 - Requirements for enforcement authority.

Code of Federal Regulations, 2014 CFR

2014-07-01

... established under § 123.34. (Clean Water Act (33 U.S.C. 1251 et seq.), Safe Drinking Water Act (42 U.S.C. 300f et seq.), Clean Air Act (42 U.S.C. 7401 et seq.), Resource Conservation and Recovery Act (42 U.S.C.... 123.27 Section 123.27 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) WATER...

40 CFR 123.27 - Requirements for enforcement authority.

Code of Federal Regulations, 2012 CFR

2012-07-01

... established under § 123.34. (Clean Water Act (33 U.S.C. 1251 et seq.), Safe Drinking Water Act (42 U.S.C. 300f et seq.), Clean Air Act (42 U.S.C. 7401 et seq.), Resource Conservation and Recovery Act (42 U.S.C.... 123.27 Section 123.27 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) WATER...

48 CFR 241.102 - Applicability.

Code of Federal Regulations, 2010 CFR

2010-10-01

... authorized by— (A) 10 U.S.C. 2394 for energy, fuels, and energy production facilities for periods not to exceed 30 years; (B) 10 U.S.C. 2394a for renewable energy for periods not to exceed 25 years; (C) 10 U.S.C. 2689 for geothermal resources that result in energy production facilities; (D) 10 U.S.C. 2809 for...

48 CFR 241.102 - Applicability.

Code of Federal Regulations, 2011 CFR

2011-10-01

... authorized by— (A) 10 U.S.C. 2394 for energy, fuels, and energy production facilities for periods not to exceed 30 years; (B) 10 U.S.C. 2394a for renewable energy for periods not to exceed 25 years; (C) 10 U.S.C. 2689 for geothermal resources that result in energy production facilities; (D) 10 U.S.C. 2809 for...

7 CFR 2.48 - Administrator, Rural Business-Cooperative Service.

Code of Federal Regulations, 2014 CFR

2014-01-01

... (33 U.S.C. 1251 et seq.); (iii) Safe Drinking Water Act, as amended (42 U.S.C. 300f et seq.); (iv... Innovation Center Demonstration program (note to 7 U.S.C. 1621). (30) Administer the Renewable Energy Systems and Energy Efficiency Improvements program (7 U.S.C. 8106). (30) Administer the renewable energy...

7 CFR 2.48 - Administrator, Rural Business-Cooperative Service.

Code of Federal Regulations, 2010 CFR

2010-01-01

... Drinking Water Act, as amended (42 U.S.C. 300f et seq.); (iv) Clean Air Act, as amended (42 U.S.C. 7401 et... Innovation Center Demonstration program (note to 7 U.S.C. 1621). (30) Administer the Renewable Energy Systems and Energy Efficiency Improvements program (7 U.S.C. 8106). (30) Administer the renewable energy...

7 CFR 2.48 - Administrator, Rural Business-Cooperative Service.

Code of Federal Regulations, 2012 CFR

2012-01-01

... Drinking Water Act, as amended (42 U.S.C. 300f et seq.); (iv) Clean Air Act, as amended (42 U.S.C. 7401 et... Innovation Center Demonstration program (note to 7 U.S.C. 1621). (30) Administer the Renewable Energy Systems and Energy Efficiency Improvements program (7 U.S.C. 8106). (30) Administer the renewable energy...

7 CFR 2.48 - Administrator, Rural Business-Cooperative Service.

Code of Federal Regulations, 2011 CFR

2011-01-01

... Drinking Water Act, as amended (42 U.S.C. 300f et seq.); (iv) Clean Air Act, as amended (42 U.S.C. 7401 et... Innovation Center Demonstration program (note to 7 U.S.C. 1621). (30) Administer the Renewable Energy Systems and Energy Efficiency Improvements program (7 U.S.C. 8106). (30) Administer the renewable energy...

7 CFR 2.48 - Administrator, Rural Business-Cooperative Service.

Code of Federal Regulations, 2013 CFR

2013-01-01

... Drinking Water Act, as amended (42 U.S.C. 300f et seq.); (iv) Clean Air Act, as amended (42 U.S.C. 7401 et... Innovation Center Demonstration program (note to 7 U.S.C. 1621). (30) Administer the Renewable Energy Systems and Energy Efficiency Improvements program (7 U.S.C. 8106). (30) Administer the renewable energy...

78 FR 17995 - Agency Information Collection Activity Under OMB Review

Federal Register 2010, 2011, 2012, 2013, 2014

2013-03-25

... collections: 49 U.S.C. Section 5337--State of Good Repair Program 49 U.S.C. Section 5339--Bus and Bus... on a quarterly basis. The information submitted ensures FTA's compliance with applicable federal laws. Title: 49 U.S.C. Section 5339--Bus and Bus Facilities Program. Abstract: 49 U.S.C. 5339--Bus and Bus...

77 FR 76075 - Whistleblower Protection Advisory Committee (WPAC)

Federal Register 2010, 2011, 2012, 2013, 2014

2012-12-26

... workers and employers, improving the investigative and enforcement process, improvements of regulations..., 49 U.S.C. 42121; the Sarbanes-Oxley Act, 18 U.S.C. 1514A; the Pipeline Safety Improvement Act, 49 U.S... Act, 6 U.S.C. 1142; the Consumer Product Safety Improvement Act, 15 U.S.C. 2087; Section 1558 of the...

50 CFR 11.2 - Scope of regulations.

Code of Federal Regulations, 2011 CFR

2011-10-01

... actions arising under the following laws and regulations issued thereunder: Lacey Act, 18 U.S.C. 43; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Bald Eagle Protection Act, 16 U.S.C. 668-668d; Endangered Species Act of 1973, 87 Stat. 884, 16 U.S.C. 1531 et seq.; and Marine Mammal Protection Act of...

50 CFR 11.2 - Scope of regulations.

Code of Federal Regulations, 2013 CFR

2013-10-01

... actions arising under the following laws and regulations issued thereunder: Lacey Act, 18 U.S.C. 43; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Bald Eagle Protection Act, 16 U.S.C. 668-668d; Endangered Species Act of 1973, 87 Stat. 884, 16 U.S.C. 1531 et seq.; and Marine Mammal Protection Act of...

50 CFR 11.2 - Scope of regulations.

Code of Federal Regulations, 2014 CFR

2014-10-01

... actions arising under the following laws and regulations issued thereunder: Lacey Act, 18 U.S.C. 43; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Bald Eagle Protection Act, 16 U.S.C. 668-668d; Endangered Species Act of 1973, 87 Stat. 884, 16 U.S.C. 1531 et seq.; and Marine Mammal Protection Act of...

50 CFR 11.2 - Scope of regulations.

Code of Federal Regulations, 2012 CFR

2012-10-01

... actions arising under the following laws and regulations issued thereunder: Lacey Act, 18 U.S.C. 43; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Bald Eagle Protection Act, 16 U.S.C. 668-668d; Endangered Species Act of 1973, 87 Stat. 884, 16 U.S.C. 1531 et seq.; and Marine Mammal Protection Act of...

50 CFR 11.2 - Scope of regulations.

Code of Federal Regulations, 2010 CFR

2010-10-01

... actions arising under the following laws and regulations issued thereunder: Lacey Act, 18 U.S.C. 43; Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq.; Bald Eagle Protection Act, 16 U.S.C. 668-668d; Endangered Species Act of 1973, 87 Stat. 884, 16 U.S.C. 1531 et seq.; and Marine Mammal Protection Act of...

50 CFR 12.23 - Administrative forfeiture proceedings.

Code of Federal Regulations, 2011 CFR

2011-10-01

... Protection Act, 16 U.S.C. 668 et seq., or Airborne Hunting Act, 16 U.S.C. 742j-1, or any wildlife or plant subject to forfeiture under the Endangered Species Act, 16 U.S.C. 1531 et seq., or any fish, wildlife or plant subject to forfeiture under the Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq., is...

50 CFR 12.23 - Administrative forfeiture proceedings.

Code of Federal Regulations, 2014 CFR

2014-10-01

... Protection Act, 16 U.S.C. 668 et seq., or Airborne Hunting Act, 16 U.S.C. 742j-1, or any wildlife or plant subject to forfeiture under the Endangered Species Act, 16 U.S.C. 1531 et seq., or any fish, wildlife or plant subject to forfeiture under the Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq., is...

50 CFR 12.23 - Administrative forfeiture proceedings.

Code of Federal Regulations, 2013 CFR

2013-10-01

... Protection Act, 16 U.S.C. 668 et seq., or Airborne Hunting Act, 16 U.S.C. 742j-1, or any wildlife or plant subject to forfeiture under the Endangered Species Act, 16 U.S.C. 1531 et seq., or any fish, wildlife or plant subject to forfeiture under the Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq., is...

50 CFR 12.23 - Administrative forfeiture proceedings.

Code of Federal Regulations, 2012 CFR

2012-10-01

... Protection Act, 16 U.S.C. 668 et seq., or Airborne Hunting Act, 16 U.S.C. 742j-1, or any wildlife or plant subject to forfeiture under the Endangered Species Act, 16 U.S.C. 1531 et seq., or any fish, wildlife or plant subject to forfeiture under the Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq., is...

15 CFR 922.152 - Prohibited or otherwise regulated activities.

Code of Federal Regulations, 2013 CFR

2013-01-01

... Protection Act, as amended, (MMPA), 16 U.S.C. 1361 et seq., the Endangered Species Act, as amended, (ESA), 16 U.S.C. 1531 et seq., and the Migratory Bird Treaty Act, as amended, (MBTA), 16 U.S.C. 703 et seq... section 312 of the Federal Water Pollution Control Act, as amended, (FWPCA), 33 U.S.C. 1322 et seq.; (C...

50 CFR 12.23 - Administrative forfeiture proceedings.

Code of Federal Regulations, 2010 CFR

2010-10-01

... Protection Act, 16 U.S.C. 668 et seq., or Airborne Hunting Act, 16 U.S.C. 742j-1, or any wildlife or plant subject to forfeiture under the Endangered Species Act, 16 U.S.C. 1531 et seq., or any fish, wildlife or plant subject to forfeiture under the Lacey Act Amendments of 1981, 16 U.S.C. 3371 et seq., is...

15 CFR 922.152 - Prohibited or otherwise regulated activities.

Code of Federal Regulations, 2012 CFR

2012-01-01

... Protection Act, as amended, (MMPA), 16 U.S.C. 1361 et seq., the Endangered Species Act, as amended, (ESA), 16 U.S.C. 1531 et seq., and the Migratory Bird Treaty Act, as amended, (MBTA), 16 U.S.C. 703 et seq... section 312 of the Federal Water Pollution Control Act, as amended, (FWPCA), 33 U.S.C. 1322 et seq.; (C...

15 CFR 922.152 - Prohibited or otherwise regulated activities.

Code of Federal Regulations, 2014 CFR

2014-01-01

... Protection Act, as amended, (MMPA), 16 U.S.C. 1361 et seq., the Endangered Species Act, as amended, (ESA), 16 U.S.C. 1531 et seq., and the Migratory Bird Treaty Act, as amended, (MBTA), 16 U.S.C. 703 et seq... section 312 of the Federal Water Pollution Control Act, as amended, (FWPCA), 33 U.S.C. 1322 et seq.; (C...

48 CFR 1552.235-71 - Treatment of confidential business information.

Code of Federal Regulations, 2013 CFR

2013-10-01

... Control Act (33 U.S.C. 1251, et seq.), the Safe Drinking Water Act (42 U.S.C. 300f et seq.), the Federal Insecticide, Fungicide, and Rodenticide Act (7 U.S.C. 136 et seq.), the Federal Food, Drug, and Cosmetic Act... Toxic Substances Control Act (15 U.S.C. 2601 et seq.). EPA regulations on confidentiality of business...

48 CFR 1552.235-71 - Treatment of confidential business information.

Code of Federal Regulations, 2011 CFR

2011-10-01

... Control Act (33 U.S.C. 1251, et seq.), the Safe Drinking Water Act (42 U.S.C. 300f et seq.), the Federal Insecticide, Fungicide, and Rodenticide Act (7 U.S.C. 136 et seq.), the Federal Food, Drug, and Cosmetic Act... Toxic Substances Control Act (15 U.S.C. 2601 et seq.). EPA regulations on confidentiality of business...

48 CFR 1552.235-71 - Treatment of confidential business information.

Code of Federal Regulations, 2014 CFR

2014-10-01

... Control Act (33 U.S.C. 1251, et seq.), the Safe Drinking Water Act (42 U.S.C. 300f et seq.), the Federal Insecticide, Fungicide, and Rodenticide Act (7 U.S.C. 136 et seq.), the Federal Food, Drug, and Cosmetic Act... Toxic Substances Control Act (15 U.S.C. 2601 et seq.). EPA regulations on confidentiality of business...

48 CFR 1552.235-71 - Treatment of confidential business information.

Code of Federal Regulations, 2012 CFR

2012-10-01

... Control Act (33 U.S.C. 1251, et seq.), the Safe Drinking Water Act (42 U.S.C. 300f et seq.), the Federal Insecticide, Fungicide, and Rodenticide Act (7 U.S.C. 136 et seq.), the Federal Food, Drug, and Cosmetic Act... Toxic Substances Control Act (15 U.S.C. 2601 et seq.). EPA regulations on confidentiality of business...