Copper and the Prion Protein: Methods, Structures, Function, and Disease

NASA Astrophysics Data System (ADS)

Millhauser, Glenn L.

2007-05-01

The transmissible spongiform encephalopathies (TSEs) arise from conversion of the membrane-bound prion protein from PrPC to PrPSc. Examples of the TSEs include mad cow disease, chronic wasting disease in deer and elk, scrapie in goats and sheep, and kuru and Creutzfeldt-Jakob disease in humans. Although the precise function of PrPC in healthy tissues is not known, recent research demonstrates that it binds Cu(II) in an unusual and highly conserved region of the protein termed the octarepeat domain. This review describes recent connections between copper and PrPC, with an emphasis on the electron paramagnetic resonance elucidation of the specific copper-binding sites, insights into PrPC function, and emerging connections between copper and prion disease.

Oxidative stress and mitochondrial dysfunction-linked neurodegenerative disorders.

PubMed

Islam, Md Torequl

2017-01-01

Reactive species play an important role in physiological functions. Overproduction of reactive species, notably reactive oxygen (ROS) and nitrogen (RNS) species along with the failure of balance by the body's antioxidant enzyme systems results in destruction of cellular structures, lipids, proteins, and genetic materials such as DNA and RNA. Moreover, the effects of reactive species on mitochondria and their metabolic processes eventually cause a rise in ROS/RNS levels, leading to oxidation of mitochondrial proteins, lipids, and DNA. Oxidative stress has been considered to be linked to the etiology of many diseases, including neurodegenerative diseases (NDDs) such as Alzheimer diseases, Amyotrophic lateral sclerosis, Friedreich's ataxia, Huntington's disease, Multiple sclerosis, and Parkinson's diseases. In addition, oxidative stress causing protein misfold may turn to other NDDs include Creutzfeldt-Jakob disease, Bovine Spongiform Encephalopathy, Kuru, Gerstmann-Straussler-Scheinker syndrome, and Fatal Familial Insomnia. An overview of the oxidative stress and mitochondrial dysfunction-linked NDDs has been summarized in this review.

[Disease concept of the slow virus infection].

PubMed

Takasu, Toshiaki

2007-08-01

This article gives a brief history of the terminology of slow virus infection, the conceptual change that occurred in it, the features common to slow infection and the current concept of slow virus infection. Björn Sigurdsson from the field of veterinary medicine proposed slow virus infection as unique mode of infection in 1954. Its initial concept was remodeled along with the general acceptance of prion theory of sheep scrapie that was proposed in 1982. The features common to slow infection include very long latency, unanimous poor prognosis, central nervous system involvement, etc. Currently the slow infection comprises those caused by slow conventional viruses that is the slow virus infection (for example subacute sclerosing panencephalitis and progressive multifocal encephalopathy in human and visna-maedi in sheep) and prion diseases (for example kuru, Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker syndrome in human, scrapie and bovine spongiform encephalopathy).

From Creutzfeldt-Jakob disease to the mad cow epidemic.

PubMed

Sternbach, G; Dibble, C L; Varon, J

1997-01-01

Hans-Gerhard Creutzfeldt and Alfons Jakob independently authored clinical and pathologic descriptions of a new syndrome in the 1920s. This syndrome, which subsequently came to be named after them, was characterized by dementia, motor and coordination abnormalities, a fatal course, and pathologic findings of diffuse spongiform neuronal degeneration. Although it appeared for many years to be little more than a medical curiosity, Creutzfeldt-Jakob disease attained widespread attention by its pathologic similarity to kuru and bovine spongiform encephalopathy, "mad cow disease." Because there are sporadic, familial, and iatrogenic forms of Creutzfeldt-Jakob disease, it is considered to have both genetic and infectious aspects. Although its causation has for some time been ascribed to "slow viruses," the etiology of Creutzfeldt-Jakob disease is currently thought to be due to prions, small proteinaceous infectious particles that have genetic encoding. The debate regarding whether the appearance of atypical Creutzfeldt-Jakob disease can be linked to the epidemic of "mad cow disease" is currently unresolved.

Molecular modelling indicates that the pathological conformations of prion proteins might be beta-helical.

PubMed Central

Downing, D T; Lazo, N D

1999-01-01

Creutzfeldt-Jakob disease, kuru, scrapie and bovine spongiform encephalopathy are diseases of the mammalian central nervous system that involve the conversion of a cellular protein into an insoluble extracellular isoform. Spectroscopic studies have shown that the precursor protein contains mainly alpha-helical and random-coil conformations, whereas the prion isoform is largely in the beta conformation. The pathogenic prion is resistant to denaturation and protease digestion and can promote the conversion of the precursor protein to the pathogenic form. These properties have yet to be explained in terms of the structural conformations of the proteins. In the present study, molecular modelling showed that prion proteins could adopt the beta-helical conformation, which has been established for a number of fibrous proteins and has been suggested previously as the basis of amyloid fibrils. The beta-helical conformation provides explanations for the biophysical and biochemical stability of prions, their ability to form templates for the transmission of pathological conformation, and the existence of phenotypical strains of the prion diseases. PMID:10510313

Geology, mineralization, and fluid inclusion study of the Kuru-Tegerek Au-Cu-Mo skarn deposit in the Middle Tien Shan, Kyrgyzstan

NASA Astrophysics Data System (ADS)

Soloviev, Serguei G.; Kryazhev, Sergey; Dvurechenskaya, Svetlana

2018-02-01

The Kuru-Tegerek Cu-Au-Mo deposit is situated in a system of Late Carboniferous subduction-related magmatic arcs of the Middle Tien Shan, which together constitute a metallogenic belt of Cu-Au-Mo (±W) porphyry, with local skarns, deposits. The deposit is related to magnetite-series gabbro-diorite to tonalite intrusion. It contains prograde magnesian and calcic skarns with abundant magnetite, associated with gabbro-diorite, and retrograde skarn with Cu mineralization, formed after intrusion of tonalite. Subsequent propylitic alteration introduced abundant chalcopyrite and pyrrhotite, and native Au culminating in zones overprinting magnetite and garnet skarn. Later quartz-muscovite-carbonate veins, formed after intrusion of late mafic quartz monzogabbro dikes, contain chalcopyrite, pyrite, arsenopyrite and other sulfides and sulfosalts, tellurides, and native Au. The earliest retrograde skarn garnet contains gaseous low-salinity (1.7-3.4 wt.% NaCl eq.) fluid inclusions homogenizing at 460-500 °C into vapor, indicating that the early fluid released from crystallizing magma was a low-density vapor. It was followed by more saline (4.0-5.0 wt.% NaCl eq.), high-temperature (400-440 °C) aqueous fluid, as fluid release from the magma progressed. Boiling of this fluid at temperatures of 420 to 370 °C and a pressure of 350-300 bar produced a low-salinity (0.6-1.2 wt.% NaCl eq.), essentially gaseous, and high-salinity (from 39 to 31 wt.% NaCl eq.) brine, with possible metal (including Cu) partitioning into both gaseous and aqueous-saline phases. Boiling was coeval with sulfide deposition in the retrograde skarn. The latest episode of the retrograde skarn stage included direct separation of saline ( 40-42 wt.% NaCl eq.) fluid from crystallizing magma. The separation of saline ( 40 to 14 wt.% NaCl eq.) fluids from a crystallizing magmatic melt continued during the propylitic stage, when fluid cooling from 370 to 320 °C, together with decreasing fO2, caused Cu and especially Au precipitation. A new influx of possibly magma-derived, low-salinity (4.5-6.7 wt.% NaCl eq.) aqueous, and then NaCl-CO2-H2O fluids, corresponds to the phyllic (quartz-muscovite-carbonate-sulfide) stage. These fluids may have a deeper source, associated with the late mafic quartz monzogabbro dikes. Fluid cooling (from 340 to 255 °C) and boiling of the NaCl-CO2-H2O fluid, together with increased fS2, increased the Au endowment.

Retardation of mobile radionuclides in granitic rock fractures by matrix diffusion

NASA Astrophysics Data System (ADS)

Hölttä, P.; Poteri, A.; Siitari-Kauppi, M.; Huittinen, N.

Transport of iodide and sodium has been studied by means of block fracture and core column experiments to evaluate the simplified radionuclide transport concept. The objectives were to examine the processes causing retention in solute transport, especially matrix diffusion, and to estimate their importance during transport in different scales and flow conditions. Block experiments were performed using a Kuru Grey granite block having a horizontally planar natural fracture. Core columns were constructed from cores drilled orthogonal to the fracture of the granite block. Several tracer tests were performed using uranine, 131I and 22Na as tracers at water flow rates 0.7-50 μL min -1. Transport of tracers was modelled by applying the advection-dispersion model based on the generalized Taylor dispersion added with matrix diffusion. Scoping calculations were combined with experiments to test the model concepts. Two different experimental configurations could be modelled applying consistent transport processes and parameters. The processes, advection-dispersion and matrix diffusion, were conceptualized with sufficient accuracy to replicate the experimental results. The effects of matrix diffusion were demonstrated on the slightly sorbing sodium and mobile iodine breakthrough curves.

Chapter 50: history of tropical neurology.

PubMed

Ogunniyi, Adesola

2010-01-01

Tropical neurology began less than two centuries ago. Consumption of dietary toxins predominated at the beginning and gave birth to the geographic entity. The story moved from lathyrism through Jamaican neuropathy to cassava-induced epidemic neuropathy, which was contrasted with Konzo, also associated with cassava. Other tropical diseases enumerated with chronological details include: Chaga's diseases, kwashiorkor, Madras type of motor neuron disease, atlanto-axial dislocation, Burkitt's lymphoma and Kuru, associated with cannibalism among the Fore linguistic group in New Guinea. More recent documentation includes the Cuban neuropathy in 1991 with an epidemic of visual loss and neuropathy, Anaphe venata entomophagy in Nigeria presenting as seasonal ataxia, and neurological aspects of the human immunodeficiency virus infection complete the picture. With time, professional associations were formed and the pioneers were given prominence. The World Federation of Neurology featured Geographic Neurology as a theme in 1977 and Tropical Neurology was given prominence at its 1989 meeting in New Delhi, India. The situation remains unchanged with regards to rare diseases like Meniere's, multiple sclerosis, hereditary disorders. However, with westernization and continued urbanization, changing disease patterns are being observed and tropical neurology may depart from dietary toxins to more western world-type disorders.

Infectious particles, stress, and induced prion amyloids

PubMed Central

2013-01-01

Transmissible encephalopathies (TSEs) are believed by many to arise by spontaneous conversion of host prion protein (PrP) into an infectious amyloid (PrP-res, PrPSc) without nucleic acid. Many TSE agents reside in the environment, with infection controlled by public health measures. These include the disappearance of kuru with the cessation of ritual cannibalism, the dramatic reduction of epidemic bovine encephalopathy (BSE) by removal of contaminated feed, and the lack of endemic scrapie in geographically isolated Australian sheep with susceptible PrP genotypes. While prion protein modeling has engendered an intense focus on common types of protein misfolding and amyloid formation in diverse organisms and diseases, the biological characteristics of infectious TSE agents, and their recognition by the host as foreign entities, raises several fundamental new directions for fruitful investigation such as: (1) unrecognized microbial agents in the environmental metagenome that may cause latent neurodegenerative disease, (2) the evolutionary social and protective functions of different amyloid proteins in diverse organisms from bacteria to mammals, and (3) amyloid formation as a beneficial innate immune response to stress (infectious and non-infectious). This innate process however, once initiated, can become unstoppable in accelerated neuronal aging. PMID:23633671

A crucial role for B cells in neuroinvasive scrapie.

PubMed

Brandner, S; Klein, M A; Aguzzi, A

1999-02-01

Although prions are most efficiently propagated via intracerebral inoculation, peripheral administration has caused kuru [Gajdusek et al, 1966], iatrogenic Creutzfeldt-Jakob disease (CJD) [Gibbs et al, 1997], bovine spongiform encephalitis (BSE), and new variant CJD [Hill et al, 1997; Bruce et al, 1997]. Neurological disease after peripheral inoculation depends on prion expansion within cells of the lymphoreticular system (LRS) [Lasmezas et al. 1996; Wilesmith et al, 1992]. In order to identify the nature of the latter cells, we inoculated a panel of immune deficient mice with prions intraperitoneally. While defects affecting only T lymphocytes had no apparent effect, all mutations affecting differentiation and responses of B lymphocytes prevented development of clinical scrapie. Since absence of B cells and of antibodies correlates with severe defects in follicular dendritic cells (FDCs), the lack of any of these three components may prevent clinical scrapie. Yet, mice expressing immunoglobulins exclusively of the M subclass without detectable specificity for PrPc, and mice with differentiated B cells but lacking functional FDCs, developed scrapie after peripheral inoculation: therefore, differentiated B cells appear to play a crucial role in neuroinvasion of scrapie regardless of B-cell receptor specificity.

Newer knowledge in comparative virology--its contribution to human health research.

PubMed

Cabasso, J J

1975-06-28

Like other comparative sciences, and despite its recent beginning comparative virology has already contributed useful applications and observations to human health research. Teachings derived from the study of Marek's disease found application in that of Burkitt's lymphoma, and may lead to a possible vaccine against the human disease. Equally useful information came from the study of canine distemper in the development of a chorio-allantoic membrane attenuated measles vaccine, and in our knowledge of subacute sclerosing panencephalitis (SSPE) of humans; from the study of reovirus-like agents of infant mice and neonatal calves in that of an acute nonbacterial gastro-enteritis of infants and young children; and from that of the cancer-producing viruses of chickens, cats, and dogs to a better understanding of some human neoplasias. Finally, Aleutian mink disease may be an excellent natural model for the study of the collagen diseases of man, and scrapie of sheep one for that of a human chronic degenerative disease of the central nervous system of humans such as Kuru. Comparative virology has proved quite productive in a relatively short period, and is unlikely to be neglected in the future.

The classical Taub-Nut system: factorization, spectrum generating algebra and solution to the equations of motion

NASA Astrophysics Data System (ADS)

Latini, Danilo; Ragnisco, Orlando

2015-05-01

The formalism of SUperSYmmetric quantum mechanics (SUSYQM) is properly modified in such a way to be suitable for the description and the solution of a classical maximally superintegrable Hamiltonian system, the so-called Taub-Nut system, associated with the Hamiltonian: In full agreement with the results recently derived by Ballesteros et al for the quantum case, we show that the classical Taub-Nut system shares a number of essential features with the Kepler system, that is just its Euclidean version arising in the limit η \\to 0, and for which a ‘SUSYQM’ approach has been recently introduced by Kuru and Negro. In particular, for positive η and negative energy the motion is always periodic; it turns out that the period depends upon η and goes to the Euclidean value as η \\to 0. Moreover, the maximal superintegrability is preserved by the η-deformation, due to the existence of a larger symmetry group related to an η-deformed Runge-Lenz vector, which ensures that in {{{R}}3} closed orbits are again ellipses. In this context, a deformed version of the third Kepler’s law is also recovered. The closing section is devoted to a discussion of the η \\lt 0 case, where new and partly unexpected features arise.

Sod1 deficiency reduces incubation time in mouse models of prion disease.

PubMed

Akhtar, Shaheen; Grizenkova, Julia; Wenborn, Adam; Hummerich, Holger; Fernandez de Marco, Mar; Brandner, Sebastian; Collinge, John; Lloyd, Sarah E

2013-01-01

Prion infections, causing neurodegenerative conditions such as Creutzfeldt-Jakob disease and kuru in humans, scrapie in sheep and BSE in cattle are characterised by prolonged and variable incubation periods that are faithfully reproduced in mouse models. Incubation time is partly determined by genetic factors including polymorphisms in the prion protein gene. Quantitative trait loci studies in mice and human genome-wide association studies have confirmed that multiple genes are involved. Candidate gene approaches have also been used and identified App, Il1-r1 and Sod1 as affecting incubation times. In this study we looked for an association between App, Il1-r1 and Sod1 representative SNPs and prion disease incubation time in the Northport heterogeneous stock of mice inoculated with the Chandler/RML prion strain. No association was seen with App, however, significant associations were seen with Il1-r1 (P = 0.02) and Sod1 (P<0.0001) suggesting that polymorphisms at these loci contribute to the natural variation observed in incubation time. Furthermore, following challenge with Chandler/RML, ME7 and MRC2 prion strains, Sod1 deficient mice showed highly significant reductions in incubation time of 20, 13 and 24%, respectively. No differences were detected in Sod1 expression or activity. Our data confirm the protective role of endogenous Sod1 in prion disease.

Molecular pathogenesis of sporadic prion diseases in man

PubMed Central

Safar, Jiri G.

2012-01-01

The yeast, fungal and mammalian prions determine heritable and infectious traits that are encoded in alternative conformations of proteins. They cause lethal sporadic, familial and infectious neurodegenerative conditions in man, including Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), kuru, sporadic fatal insomnia (SFI) and likely variable protease-sensitive prionopathy (VPSPr). The most prevalent of human prion diseases is sporadic (s)CJD. Recent advances in amplification and detection of prions led to considerable optimism that early and possibly preclinical diagnosis and therapy might become a reality. Although several drugs have already been tested in small numbers of sCJD patients, there is no clear evidence of any agent’s efficacy. Therefore, it remains crucial to determine the full spectrum of sCJD prion strains and the conformational features in the pathogenic human prion protein governing replication of sCJD prions. Research in this direction is essential for the rational development of diagnostic as well as therapeutic strategies. Moreover, there is growing recognition that fundamental processes involved in human prion propagation – intercellular induction of protein misfolding and seeded aggregation of misfolded host proteins – are of far wider significance. This insight leads to new avenues of research in the ever-widening spectrum of age-related human neurodegenerative diseases that are caused by protein misfolding and that pose a major challenge for healthcare. PMID:22421210

Gerstmann-Straeussler-Scheinker disease with P102L prion protein gene mutation presenting with rapidly progressive clinical course.

PubMed

Iwasaki, Yasushi; Mori, Keiko; Ito, Masumi; Nokura, Kazuya; Tatsumi, Shinsui; Mimuro, Maya; Kitamoto, Tetsuyuki; Yoshida, Mari

2014-01-01

We describe an autopsied case of a Japanese woman with Gerstmann-Straeussler-Scheinker disease (GSS) presenting with a rapidly progressive clinical course. Disease onset occurred at the age of 54 with dementia and gait disturbance. Her clinical course progressively deteriorated until she reached a bedridden state with myoclonus 9 months after onset. Two months later, she reached the akinetic mutism state. Nasal tube feeding was introduced at this point and continued for several years. Electroencephalograms showed diffuse slowing without periodic sharp-wave complexes. Diffusion-weighted magnetic resonance imaging (MRI) showed widespread cerebral cortical hyperintensity. Prion protein (PrP) gene analysis revealed a Pro to Leu point mutation at codon 102 with methionine homozygosity at codon 129. The patient died of respiratory failure after a total disease duration of 62 months. Neuropathologic examination revealed widespread spongiform change with numerous eosinophilic amyloid plaques (Kuru plaques) in the cerebral and cerebellar cortices by H & E staining. Diffuse myelin pallor with axon loss of the cerebral white matter, suggestive of panencephalopathic-type pathology was observed. Numerous PrP immunopositive plaques and diffuse synaptic-type PrP deposition were extensively observed, particularly in the cerebral and cerebellar cortices. Western blot analysis of proteinase Kresistant PrP showed a characteristic band pattern with a small molecular band of 6 kDa. The reason for the similarity in clinicopathologic findings between the present case and Creutzfeldt-Jakob disease is uncertain; however, the existence of an unknown disease-modifying factor is suspected.

Molecular mechanisms of chronic wasting disease prion propagation

PubMed Central

Moreno, Julie A.; Telling, Glenn C.

2018-01-01

Prion disease epidemics, which have been unpredictable recurrences, are of significant concern for animal and human health. Examples include kuru, once the leading cause of death among the Fore people in Papua New Guinea and caused by mortuary feasting; bovine spongiform encephalopathy (BSE) and its subsequent transmission to humans in the form of variant Creutzfeldt-Jakob disease (vCJD); and repeated examples of large-scale prion disease epidemics in animals caused by contaminated vaccines. The etiology of chronic wasting disease (CWD), a relatively new and burgeoning prion epidemic in deer, elk, and moose (members of the cervid family), is more enigmatic. The disease was first described in captive and later in wild mule deer and subsequently in free-ranging as well as captive Rocky Mountain elk, white-tailed deer, and most recently moose. It is therefore the only recognized prion disorder of both wild and captive animals. In addition to its expanding range of hosts, CWD continues to spread to new geographical areas, including recent cases in Norway. The unparalleled efficiency of the contagious transmission of the disease combined with high densities of deer in certain areas of North America complicates strategies for controlling CWD and raises concerns about its potential spread to new species. Because there is a high prevalence of CWD in deer and elk, which are commonly hunted and consumed by humans, the possibility of zoonotic transmission is particularly concerning. Here we review the current status of naturally occurring CWD and describe advances in our understanding of its molecular pathogenesis, as shown by studies of CWD prions in novel in vivo and in vitro systems. PMID:28193766

Recombinant human prion protein fragment 90-231, a useful model to study prion neurotoxicity.

PubMed

Corsaro, Alessandro; Thellung, Stefano; Villa, Valentina; Nizzari, Mario; Aceto, Antonio; Florio, Tullio

2012-01-01

Transmissible spongiform encephalopathies (TSE), or prion diseases, are a group of fatal neurodegenerative disorders of animals and humans. Human diseases include Creutzfeldt-Jakob (CJD) and Gerstmann-Straussler-Scheinker (GSSD) diseases, fatal familial insomnia, and Kuru. Human and animal TSEs share a common histopathology with a pathognomonic triad: spongiform vacuolation of the grey matter, neuronal death, glial proliferation, and, more inconstantly, amyloid deposition. According to the "protein only" hypothesis, TSEs are caused by a unique post-translational conversion of normal, host-encoded, protease-sensitive prion protein (PrP(sen) or PrP(C)) to an abnormal disease-associated isoform (PrP(res) or PrP(Sc)). To investigate the molecular mechanism of neurotoxicity induced by PrP(Sc) we developed a protocol to obtain millimolar amounts of soluble recombinant polypeptide encompassing the amino acid sequence 90-231 of human PrP (hPrP90-231). This protein corresponds to the protease-resistant prion protein fragment that originates after amino-terminal truncation. Importantly, hPrP90-231 has a flexible backbone that, similar to PrP(C), can undergo to structural rearrangement. This peptide, structurally resembling PrP(C), can be converted in a PrP(Sc)-like conformation, and thus represents a valuable model to study prion neurotoxicity. In this article we summarized our experimental evidence on the molecular and structural mechanisms responsible of hPrP90-231 neurotoxicity on neuroectodermal cell line SHSY5Y and the effects of some PrP pathogen mutations identified in familial TSE.

Hidden treasures - 50 km points of interests

NASA Astrophysics Data System (ADS)

Lommi, Matias; Kortelainen, Jaana

2015-04-01

Tampere is third largest city in Finland and a regional centre. During 70's there occurred several communal mergers. Nowadays this local area has both strong and diversed identity - from wilderness and agricultural fields to high density city living. Outside the city center there are interesting geological points unknown for modern city settlers. There is even a local proverb, "Go abroad to Teisko!". That is the area the Hidden Treasures -student project is focused on. Our school Tammerkoski Upper Secondary School (or Gymnasium) has emphasis on visual arts. We are going to offer our art students scientific and artistic experiences and knowledge about the hidden treasures of Teisko area and involve the Teisko inhabitants into this project. Hidden treasures - Precambrian subduction zone and a volcanism belt with dense bed of gold (Au) and arsenic (As), operating goldmines and quarries of minerals and metamorphic slates. - North of subduction zone a homogenic precambrian magmastone area with quarries, products known as Kuru Grey. - Former ashores of post-glasial Lake Näsijärvi and it's sediments enabled the developing agriculture and sustained settlement. Nowadays these ashores have both scenery and biodiversity values. - Old cattle sheds and dairy buildings made of local granite stones related to cultural stonebuilding inheritance. - Local active community of Kapee, about 100 inhabitants. Students will discover information of these "hidden" phenomena, and rendering this information trough Enviromental Art Method. Final form of this project will be published in several artistic and informative geocaches. These caches are achieved by a GPS-based special Hidden Treasures Cycling Route and by a website guiding people to find these hidden points of interests.

Human prion diseases: surgical lessons learned from iatrogenic prion transmission.

PubMed

Bonda, David J; Manjila, Sunil; Mehndiratta, Prachi; Khan, Fahd; Miller, Benjamin R; Onwuzulike, Kaine; Puoti, Gianfranco; Cohen, Mark L; Schonberger, Lawrence B; Cali, Ignazio

2016-07-01

The human prion diseases, or transmissible spongiform encephalopathies, have captivated our imaginations since their discovery in the Fore linguistic group in Papua New Guinea in the 1950s. The mysterious and poorly understood "infectious protein" has become somewhat of a household name in many regions across the globe. From bovine spongiform encephalopathy (BSE), commonly identified as mad cow disease, to endocannibalism, media outlets have capitalized on these devastatingly fatal neurological conditions. Interestingly, since their discovery, there have been more than 492 incidents of iatrogenic transmission of prion diseases, largely resulting from prion-contaminated growth hormone and dura mater grafts. Although fewer than 9 cases of probable iatrogenic neurosurgical cases of Creutzfeldt-Jakob disease (CJD) have been reported worldwide, the likelihood of some missed cases and the potential for prion transmission by neurosurgery create considerable concern. Laboratory studies indicate that standard decontamination and sterilization procedures may be insufficient to completely remove infectivity from prion-contaminated instruments. In this unfortunate event, the instruments may transmit the prion disease to others. Much caution therefore should be taken in the absence of strong evidence against the presence of a prion disease in a neurosurgical patient. While the Centers for Disease Control and Prevention (CDC) and World Health Organization (WHO) have devised risk assessment and decontamination protocols for the prevention of iatrogenic transmission of the prion diseases, incidents of possible exposure to prions have unfortunately occurred in the United States. In this article, the authors outline the historical discoveries that led from kuru to the identification and isolation of the pathological prion proteins in addition to providing a brief description of human prion diseases and iatrogenic forms of CJD, a brief history of prion disease nosocomial transmission, and a summary of the CDC and WHO guidelines for prevention of prion disease transmission and decontamination of prion-contaminated neurosurgical instruments.

Human prion diseases: surgical lessons learned from iatrogenic prion transmission

PubMed Central

Bonda, David J.; Manjila, Sunil; Mehndiratta, Prachi; Khan, Fahd; Miller, Benjamin R.; Onwuzulike, Kaine; Puoti, Gianfranco; Cohen, Mark L.; Schonberger, Lawrence B.; Cali, Ignazio

2016-01-01

The human prion diseases, or transmissible spongiform encephalopathies, have captivated our imaginations since their discovery in the Fore linguistic group in Papua New Guinea in the 1950s. The mysterious and poorly understood “infectious protein” has become somewhat of a household name in many regions across the globe. From bovine spongiform encephalopathy (BSE), commonly identified as mad cow disease, to endocannibalism, media outlets have capitalized on these devastatingly fatal neurological conditions. Interestingly, since their discovery, there have been more than 492 incidents of iatrogenic transmission of prion diseases, largely resulting from prion-contaminated growth hormone and dura mater grafts. Although fewer than 9 cases of probable iatrogenic neurosurgical cases of Creutzfeldt-Jakob disease (CJD) have been reported worldwide, the likelihood of some missed cases and the potential for prion transmission by neurosurgery create considerable concern. Laboratory studies indicate that standard decontamination and sterilization procedures may be insufficient to completely remove infectivity from prion-contaminated instruments. In this unfortunate event, the instruments may transmit the prion disease to others. Much caution therefore should be taken in the absence of strong evidence against the presence of a prion disease in a neurosurgical patient. While the Centers for Disease Control and Prevention (CDC) and World Health Organization (WHO) have devised risk assessment and decontamination protocols for the prevention of iatrogenic transmission of the prion diseases, incidents of possible exposure to prions have unfortunately occurred in the United States. In this article, the authors outline the historical discoveries that led from kuru to the identification and isolation of the pathological prion proteins in addition to providing a brief description of human prion diseases and iatrogenic forms of CJD, a brief history of prion disease nosocomial transmission, and a summary of the CDC and WHO guidelines for prevention of prion disease transmission and decontamination of prion-contaminated neurosurgical instruments. PMID:27364252

Along-Strike Differences of the Main Himalayan Thrust and Deformation within the Indian Crust: Insights from Seismicity and Seismic Velocities in Bhutan and its Foreland

NASA Astrophysics Data System (ADS)

Diehl, T.; Singer, J.; Hetényi, G.; Kissling, E. H.; Clinton, J. F.

2015-12-01

The seismicity of Bhutan is characterized by the apparent lack of great earthquakes and a significantly lower activity compared to most other parts of the Himalayan arc. To better understand the underlying mechanisms of this anomalously low activity and to relate it with possible along-strike differences in the structure of the orogenic belt, a temporary network with up to 38 broadband seismometers was installed in Bhutan between January 2013 and November 2014. In this work we present a catalog of local and regional earthquakes detected and located with the GANSSER network complemented by regional stations in India, Bangladesh, and China. State-of-the-art data analysis and earthquake location procedures were applied to derive a high-precision earthquake catalog of Bhutan and surrounding regions. Focal mechanisms from regional moment tensor inversions and first-motion polarities complement the earthquake catalog. In the vicinity of the Shumar-Kuru Chu Spur in East Bhutan, seismicity forms a moderately dipping structure at about 12 km depth, which we associate with the Main Himalayan Thrust (MHT). North of 27.6°N the dip of the structure steepens, which can be interpreted as a ramp along the MHT. In West Bhutan seismicity occurs at depths of 20 to 40 km and receiver function images indicate that seismicity occurs in the underthrusting Indian crust rather than on the MHT. The highest seismic activity is clustered along the Goalpara Lineament, a dextral NE-SW striking shear zone in southwest Bhutan, which appears to connect to the western edge of the Shillong Plateau in the foreland. Focal depths indicate that this shear zone is located at depths of 20-30 km and therefore in the underthrusting Indian crust. Preliminary results of a 3D local earthquake tomography show substantial differences in the uppermost crust between east and west Bhutan. Consistent with our receiver function images, the results also indicate a thinning of the crustal root towards eastern Bhutan.

Distinct pathological phenotypes of Creutzfeldt-Jakob disease in recipients of prion-contaminated growth hormone.

PubMed

Cali, Ignazio; Miller, Cathleen J; Parisi, Joseph E; Geschwind, Michael D; Gambetti, Pierluigi; Schonberger, Lawrence B

2015-06-25

The present study compares the clinical, pathological and molecular features of a United States (US) case of growth hormone (GH)-associated Creutzfeldt-Jakob disease (GH-CJD) (index case) to those of two earlier referred US cases of GH-CJD and one case of dura mater (d)-associated CJD (dCJD). All iatrogenic CJD (iCJD) subjects were methionine (M) homozygous at codon 129 (129MM) of the prion protein (PrP) gene and had scrapie prion protein (PrP(Sc)) type 1 (iCJDMM1). The index subject presented with ataxia, weight loss and changes in the sleep pattern about 38 years after the midpoint of GH treatment. Autopsy examination revealed a neuropathological phenotype reminiscent of both sCJDMV2-K (a sporadic CJD subtype in subjects methionine/valine heterozygous at codon 129 with PrP(Sc) type 2 and the presence of kuru plaques) and variant CJD (vCJD). The two earlier cases of GH-CJDMM1 and the one of dCJDMM1 were associated with neuropathological phenotypes that differed from that of the index case mainly because they lacked PrP plaques. The phenotype of the earlier GH-CJDMM1 cases shared several, but not all, characteristics with sCJDMM1, whereas dCJDMM1 was phenotypically indistinguishable from sCJDMM1. Two distinct groups of dCJDMM1 have also been described in Japan based on clinical features, the presence or absence of PrP plaques and distinct PK-resistant PrP(Sc) (resPrP(Sc)) electrophoretic mobilities. The resPrP(Sc) electrophoretic mobility was, however, identical in our GH-CJDMM1 and dCJDMM1 cases, and matched that of sCJDMM1. Our study shows that receipt of prion-contaminated GH can lead to a prion disease with molecular features (129MM and PrP(Sc) type 2) and phenotypic characteristics that differ from those of sporadic prion disease (sCJDMM1), a difference that may reflect adaptation of "heterologous" prion strains to the 129MM background.

[Doctor Francoise Cathala and history of prions diseases].

PubMed

Court, L; Hauw, J-J

2015-12-01

Doctor Françoise Cathala Pagesy, MD, MS, born on July 7, 1921 in Paris, passed away peacefully at home on November 5, 2012. Unconventional, passionate and enthusiastic neurologist and virologist, she devoted her life to research on latent and slow viral infections, specializing mainly on unconventional transmissible agents or prions. As a research member of Inserm (French Institute for Medical Research), she soon joined the team of Carlton Gajdusek (the NINCDS - National Institute of Nervous Central System and Stroke - of NIH), who first demonstrated the transmissibility of kuru and Creutzfeldt-Jakob disease to monkeys. When she came back to Paris, where she was followed by one of NIH members, Paul Brown, she joined the Centre de Recherches du Service de Santé des Armées (Army Health Research Center), in Percy-Clamart, where she found the experimental design and the attentive help needed for her research, which appeared heretical to many French virologists, including some authorities. A large number of research programs were set up with numerous collaborations involving CEA (Center for Atomic Energy) and other institutions in Paris and Marseilles on epidemiology, results of tissue inoculation, electrophysiology and neuropathology of human and animal prions diseases, and resistance of the infectious agent. International symposia were set up, where met, in the Val-de-Grâce hospital in Paris, the research community on "slow viral diseases". Stanley Prusiner introduced the concept - then badly accepted and still in evolution - of prion, a protein only infectious agent. Before retiring from Inserm, Françoise Cathala predicted and was involved in some of the huge sanitary crises in France. These were, first, Creutzfeldt-Jakob disease from contaminated growth hormone extracted from cadavers, which led parents to instigate legal procedure - a quite unusual practice in France. The second was Mad cow disease in the United Kingdom then in France, followed by new variant of Creutzfeldt-Jakob human epidemics, paradigmatic food safety crisis bringing together the poles of production (beef and meat-and-bone meal) and consumption, and leading to an unexpected social bang. Through Françoise Cathala exemplary life, the history of French, and more generally of worldwide prions diseases is dealt with. Copyright © 2014. Published by Elsevier Masson SAS.

PREFACE: The 5th International Symposium in Quantum Theory and Symmetries (QTS5)

NASA Astrophysics Data System (ADS)

Arratia, O.; Calzada, J. A.; Gómez-Cubillo, F.; Negro, J.; del Olmo, M. A.

2008-02-01

This volume of Journal of Physics: Conference Series contains the Proceedings of the 5th International Symposium in Quantum Theory and Symmetries (QTS5), held in Valladolid, Spain, 22-28 July 2007. This is the fifth of a series of conferences previously held in Goslar (Germany) 1999, QTS1; Cracow (Poland) 2001, QTS2; Cincinnati (USA) 2003, QTS3, and Varna (Bulgaria) 2005, QTS4. The QTS5 symposium gathered 181 participants from 39 countries working in different fields on Theoretical Physics. The spirit of the QTS conference series is to join researchers in a wide variety of topics in Theoretical Physics, as a way to make accessible recent results and the new lines of different fields. The QTS5 conference offered the following list of topics: Symmetries in String Theory, Quantum Gravity and related Symmetries in Quantum Field Theories, Conformal and Related Field Theories, Lattice and Noncommutative Theories, Gauge Theories Quantum Computing, Information and Control Foundations of Quantum Theory Quantum Optics, Coherent States, Wigner Functions Dynamical and Integrable Systems Symmetries in Condensed Matter and Statistical Physics Symmetries in Particle Physics, Nuclear, Atomic and Molecular Nonlinear Quantum Mechanics Time Asymmetric Quantum Mechanics SUSY Quantum Mechanics, PT symmetries and pseudo-Hamiltonians Mathematical Methods for Symmetries and Quantum Theories Symmetries in Chemistry Biology and other Sciences Papers accepted for publication in the present issue are based on the contributions from the participants in the QTS5 conference after a peer review process. In addition, a special issue of Journal Physics A: Mathematical and Theoretical contains contributions from plenary speakers, some participants as well as contributions from other authors whose works fit into the topics of the conference. The organization of the conference had the following pattern. In the morning there were five plenary or general sessions for all the participants, which aimed to give a state of the art view of different topics. The afternoon was devoted to 6 parallel sessions, each one including the most afine topics. Also there was a poster session that could be visited in the intermediates and during the coffe breaks. A total of 23 plenary conferences, 130 parallel communications and 25 posters were presented in QTS5. All the sessions were given in the Palace of Conferences Conde Ansúrez which among other facilities also had a computer room. As social activities we had a guided walk around the historical part of the city, a visit to the nearby city of Segovia, as well as a welcome party and farewell dinner. These activities were intended to foster contact and informal discussions among the participants in the meeting. We are much indebted to several institutions that without their support the organization of the QTS5 symposium would not have been possible. In this respect we greatly acknowledge to Ministerio de Educación of Spain and Junta de Castilla y León for general financial support, to Fundación Universidades de Castilla y León for a number of grants to young researchers who otherwise would not have attended the conference, to the European Physical Society that provided a number of grants for eastern countries and to the University of Valladolid where the event took place. We want to thank all the members of the Conference Board and the International Advisory Committee of QTS5 for honoring us with their help and support. Here we must mention our gratitude to the other members of the Local Organizing Committe of QTS5, Manuel Gadella, José Manuel Izquierdo and Sengül Kuru, who are not Editors of this JPCS issue but shared with us the complex organization of this event. We acknowledge IOP Publishing for the facilities in the publication of these Proceedings. Finally, on behalf of the Local Organizing Committee, we would like to thank all the participants in the QTS5 conference for their presence, contributions, and for the good atmosphere achieved during their stay. We hope that the experience of spending these days in Valladolid has been most fruitful for all of them. O Arratia, J A Calzada, F Gómez-Cubillo, J Negro and M A del Olmo Universidad de Valladolid, Spain Editors of the QTS5 Proceedings Conference Board S T Ali (Montreal) L L Boyle (Canterbury) M A del Olmo (Valladolid) V K Dobrev (Sofia) H D Doebner (Clausthal), Chair E Kapuscik (Cracow) V I Man'ko (Moscow) G Marmo (Naples) G S Pogosyan (Yerevan and Dubna) T H Seligman (Cuernavaca) A I Solomon (Paris and Open University) P Suranyi (Cincinnati) L C R Wijewardhana (Cincinnati) International Advisory Committee L Accardi, (Roma) M Asorey, (Zaragoza) M T Batchelor, (Canberra) C M Bender, (Washington) A Bohm, (Texas) E Celeghini, (Firenze) I Cirac, (Garching) S Ferrara, (CERN) J P Gazeau, (Paris) G Goldin , (Rutgers) F Iachello, (Yale) T Janssen, (Nijmegen) J Klauder, (Gainesville) P Kulish, (St Petersburg) B Mielnik, (Mexico DF) W Miller, (Minneapolis) M Plyushchay, (Santiago de Chile) O Ragnisco, (Roma) S Randjbar-Daemi, (ICTP) M Santander, (Valladolid) G Sierra, (Madrid) P Townsend, (Cambridge) S Twarock, (York) F Wilczek, (Boston) P Winternitz, (Montreal) K B Wolf, (Cuernavaca) Local Organizing Committee (University of Valladolid) Oscar Arratia Juan A Calzada Manuel Gadella Fernando Gómez-Cubillo José Manuel Izquierdo Sengül Kuru Javier Negro Mariano A del Olmo (Chairman) Official photograph

Sorption and diffusion of selenium oxyanions in granitic rock

NASA Astrophysics Data System (ADS)

Ikonen, Jussi; Voutilainen, Mikko; Söderlund, Mervi; Jokelainen, Lalli; Siitari-Kauppi, Marja; Martin, Andrew

2016-09-01

The processes controlling diffusion and sorption of radionuclides have been studied extensively in the laboratory, whereas, only a few in-situ experiments have been carried out in order to study in-situ diffusion over the long-term (several years). This is largely due to the fact that in-situ experiments are typically time consuming and cost intensive, and it is commonly accepted that laboratory scale tests are well-established approaches to characterizing the properties of geological media. In order to assess the relevance of laboratory experiments, the Swiss National Cooperative for Disposal of Radioactive Waste (Nagra) have been conducting extensive experiments in the Underground Rock Laboratory (URL) at the Grimsel Test Site (GTS) in order to study radionuclide transport and retention in-situ. One of the elements used in these experiments is non-radioactive selenium, as an analog for the radiotoxic isotope Se-79, which is present in radioactive waste. In this work, two laboratory through-diffusion experiments using selenium as a tracer were carried out in block (decimeter) scale rock specimens to support one of the ongoing radionuclide transport and retention in-situ experiment at the GTS mentioned above. The though-diffusion tests of selenium were performed under atmospheric conditions in both Kuru grey granite (KGG) and Grimsel granodiorite (GG). The decrease of selenium concentration in an inlet hole drilled into each of the rock samples and the breakthrough of selenium into sampling holes drilled around the inlet were analyzed using Inductively Coupled Plasma Mass Spectrometry (ICP-MS). The effective diffusion (De) and distribution coefficients (Kd) of selenium were then determined from the changes of selenium concentration in the inlet and sampling holes using a Time-Domain Diffusion (TDD) simulations. In addition, Kd of selenium was measured by batch sorption experiments as a function of pH and Se concentration in atmospheric conditions and nitrogen atmosphere. The speciation of selenium was studied by HPLC-ICP-MS in simulated ground waters of each of the rock types. The Kd of selenium was found to be in the range of (6.2-7.0 ± 2.0) × 10- 3 m3/kg in crushed rock whereas the Kd obtained from block scale through diffusion experiment varied between (1.5 ± 0.3) × 10- 3 m3/kg and (1.0 ± 0.6) × 10- 4 m3/kg. The De of selenium was significantly higher for GG; De = (2.5 ± 1.5) × 10- 12 m2/s than for KGG; De = (7 ± 2) × 10- 13 m2/s due to the higher permeability of GG compared with KGG.

Sorption and diffusion of selenium oxyanions in granitic rock.

PubMed

Ikonen, Jussi; Voutilainen, Mikko; Söderlund, Mervi; Jokelainen, Lalli; Siitari-Kauppi, Marja; Martin, Andrew

2016-09-01

The processes controlling diffusion and sorption of radionuclides have been studied extensively in the laboratory, whereas, only a few in-situ experiments have been carried out in order to study in-situ diffusion over the long-term (several years). This is largely due to the fact that in-situ experiments are typically time consuming and cost intensive, and it is commonly accepted that laboratory scale tests are well-established approaches to characterizing the properties of geological media. In order to assess the relevance of laboratory experiments, the Swiss National Cooperative for Disposal of Radioactive Waste (Nagra) have been conducting extensive experiments in the Underground Rock Laboratory (URL) at the Grimsel Test Site (GTS) in order to study radionuclide transport and retention in-situ. One of the elements used in these experiments is non-radioactive selenium, as an analog for the radiotoxic isotope Se-79, which is present in radioactive waste. In this work, two laboratory through-diffusion experiments using selenium as a tracer were carried out in block (decimeter) scale rock specimens to support one of the ongoing radionuclide transport and retention in-situ experiment at the GTS mentioned above. The though-diffusion tests of selenium were performed under atmospheric conditions in both Kuru grey granite (KGG) and Grimsel granodiorite (GG). The decrease of selenium concentration in an inlet hole drilled into each of the rock samples and the breakthrough of selenium into sampling holes drilled around the inlet were analyzed using Inductively Coupled Plasma Mass Spectrometry (ICP-MS). The effective diffusion (De) and distribution coefficients (Kd) of selenium were then determined from the changes of selenium concentration in the inlet and sampling holes using a Time-Domain Diffusion (TDD) simulations. In addition, Kd of selenium was measured by batch sorption experiments as a function of pH and Se concentration in atmospheric conditions and nitrogen atmosphere. The speciation of selenium was studied by HPLC-ICP-MS in simulated ground waters of each of the rock types. The Kd of selenium was found to be in the range of (6.2-7.0±2.0)×10(-3)m(3)/kg in crushed rock whereas the Kd obtained from block scale through diffusion experiment varied between (1.5±0.3)×10(-3)m(3)/kg and (1.0±0.6)×10(-4)m(3)/kg. The De of selenium was significantly higher for GG; De=(2.5±1.5)×10(-12)m(2)/s than for KGG; De=(7±2)×10(-13)m(2)/s due to the higher permeability of GG compared with KGG. Copyright © 2016 Elsevier B.V. All rights reserved.

Effect of Microwave Pre-Processing of Pelletized Biomass on its Gasification and Combustion / Mikroviļnu Priekšapstrādes Ietekme Uz Granulētas Biomasas Gazifikācijas Un Degšanas Procesiem

NASA Astrophysics Data System (ADS)

Barmina, I.; Līckrastiņa, A.; Valdmanis, J.; Valdmanis, R.; Zaķe, M.; Arshanitsa, A.; Telysheva, G.; Solodovnik, V.

2013-08-01

To effectively produce clean heat energy from biomass, microwave (mw) pre-processing of its different types - pelletized wood (spruce), herbaceous biomass (reed canary grass) and their mixture (50:50) - was carried out at the 2.45 GHz frequency with different durations of biomass exposure to high-frequency oscillations. To estimate the mw pre-processing effect on the structure, composition and fuel characteristics of biomass, its thermogravimetric (TG), infrared spectroscopy (FTIR) measurements and elemental analysis were made. The pre-processing is shown to enhance the release of moisture and low-calorific volatiles and the partial destruction of biomass constituents (hemicelluloses, cellulose), promoting variations in the elemental composition and heating values of biomass. The field-enhanced variations of biomass characteristics and their influence on its gasification and combustion were studied using an integrated system of a biomass gasifier and a combustor with swirl-enhanced stabilization of the flame reaction zone. The results show that the mw pre-processing of biomass pellets provides a faster weight loss at the gasification, and, therefore, faster ignition and combustion of the activated pellets along with increased output of heat energy at their burnout Veikti kompleksi eksperimentālie pētījumi par mikroviļņu (2,45 GHz) priekšapstrādes ietekmi uz dažādas izcelsmes biomasas granulu (egles, miežabrāļa un to maisījumu 50:50) gazifikācijas un degšanas procesiem. Pētījumi apvieno granulētās biomasas elementārā sastāva un termogravimetriskos mērījumus, kā arī granulētās biomasas gazifikācijas un degšanas procesu kompleksu izpēti, apvienojot biomasas svara izmaiņu kinētiskos mērījumus ar degšanas zonas temperatūras, iekārtas jaudas un degšanas produktu sastāva kinētiskiem mērījumiem. Pētījumiem izmantota mazas jaudas eksperimentālā iekārta (līdz 2,5 kW), kuru veido integrēts gazifikātors un degšanas kamera. Pētījumu rezultātā konstatēts, ka mikroviļņu priekšapstrāde nodrošina intensīvāku biomasas gazifikāciju, ātrāku gaistošo savienojumu veidošanos, uzliesmošanu un pilnīgāku sadedzināšanu ar sekojošu saražotās īpatnējā siltuma enerģijas pieaugumu

Receiving And Data Acquisition Systems Of Rt-32 For Vlbi Observations / Rt-32 Uztveršanas Un Datu Reģistrācijas Sistēmas Vlbi Novērojumiem

NASA Astrophysics Data System (ADS)

Bezrukovs, Vl.; Shmeld, I.; Nechaeva, M.; Trokss, J.; Bezrukovs, D.; Klapers, M.; Berzins, A.; Lesins, A.; Dugin, N.

2012-12-01

Radiotelescope RT-32 is a fully steerable 32-m parabolic antenna located at Irbene and belonging to Ventspils International Radio Astronomy Centre (VIRAC). Currently, the work on upgrading and repair of its receiving hardware and data acquisition systems is of high priority for the VIRAC. One of the main scientific objectives for the VIRAC Radioastronomical observatory is VLBI (very long baseline interferometry) observations in centimetre wavelengths in collaboration with world VLBI networks, such as European VLBI network (EVN), Low Frequency VLBI network (LFVN), and others. During the last years the room in the secondary focus of telescope was reconstructed, and several new receivers were installed. Currently, RT-32 observations are carried out in four different bands: 92 cm, 18 cm, 6 cm, and 2.5 cm. First three of them are already successfully employed in diversified VLBI experiments. The receiver on 2.5 cm band has only one linear polarized chain and is used mainly for the methanol maser single dish observations. The apparatus system of RT-32 is equipped with two independent VLBI data acquisition systems: TN-16, and DBBC in combination with MK5b. Both systems are employed in interferometric observations depending on the purpose of experiment and the enabled radiotelescopes. The current status of RT-32, the availability of its receiving and data acquisition units for VLBI observations and the previous VLBI sessions are discussed. Radioteleskops RT-32 ir Ventspils Starptautiskajam Radioastronomijas Centram (VSRC) piederoša pilnas piedziņas 32 m diametra paraboliskā antena. Pašreiz visaktuālākie VSRC veicamie darbi ir saistīti ar RT-32 uztverošās aparatūras un datu reģistrēšanas sistēmas labošanu un modernizāciju. Viens no radioastronomiskās observatorijas galvenajiem zinātniskajiem uzdevumiem ir sevišķi lielas bāzes interferometriskie (VLBI) novērojumi centimetru viļņu garumu diapazonā sadarbībā ar pasaules VLBI tīkla partneriem, tādiem kā Eiropas VLBI tīkls, Zemo frekvenču VLBI tīkls (LFVN) un citiem. Pēdējos gados rekonstruēta teleskopa sekundārajā fokusā izvietotā uztvērēju telpa un tajā uzstādīti vairāki jauni uztvērēji. Pašreiz radioteleskops ļauj veikt novērojumus četros viļņu garumu diapazonos: 92 cm, 18 cm, 6 cm un 2.5 cm. No minētajiem pirmie 3 jau tiek veiksmīgi izmantoti dažādos VLBI eksperimentos. 2.5 cm uztvērējam ir tikai viens lineārās polarizācijas kanāls, kuru izmanto galvenokārt metanola māzeru novērojumiem viena teleskopa režīmā. RT-32 aparatūru veido divas neatkarīgas VLBI datu reģistrācijas sistēmas: TN-16 un DBBC kopā ar Mark5b. Abas sistēmas izmanto interferometriskajos novērojumos atkarībā no eksperimentu mērķa un radioteleskopa iespējām. Aplūkots Irbenes RT-32 radioteleskopa pašreizējais statuss, tā VLBI novērojumiem piemērotās uztveršanas un datu reģistrācijas iekārtas, kā arī notikušajās VLBI sesijās uzkrātā pieredze.

Determination of Two-Liquid Mixture Composition by Assessing Dielectric Parameters 1. Precise Measuring System / Divu Šķidrumu Maisījuma Sastāva Noteikšana, Izvērtējot to Dielektriskos Parametrus 1. Precīza Mērīšanas Sistēma

NASA Astrophysics Data System (ADS)

Vilitis, O.; Shipkovs, P.; Merkulovs, D.

2013-08-01

Concentration measurements are important in bioethanol industries, in the R&D areas, for chemical, medical and microbiological analyses and processing as well as for diagnostics, manufacturing, etc. The overview shows development of the structural design of a system for measuring the concentration of solutions and mixtures consisting of two dielectric liquids. The basic principles of the system's design are given along with relevant equations. The concentration of dielectric liquids is measured using devices with capacitive sensors (1-300 pF). The operational frequency of the developed measuring system is 100.000 kHz. Configuration of the system excludes some errors usually arising at measurements, and broadens its applicability. For testing, the system was calibrated for measuring the concentration of anhydrous ethanol + de-ionized water mixture. Experimental results have shown a stable resolution of ±0.005 pF at measuring the sensor capacitance and a reproducible resolution better than ±0.01% at measuring the ethanol volume concentration Rakstā esam parādījuši iespējas izveidot augstas precizitātes, kompaktu, lētu un ērtu lietošanai dielektrisku šķidrumu mērīšanas sistēmu koncentrācijas noteikšanai. Šī sistēma ir piemērojama kapacitīviem sensoriem, kuru kapacitāte ir atkarīga no sensora izveidojuma kā arī mērāmā šķidruma dielektriskās konstantes vērtības, un kapacitāte var tikt noteikta pie frekvences 100,000 kHz robežās no 1 F līdz 300 pF. Mērīšanas sistēmas pārbaudei, sistēma tika kalibrēta etanola koncentrācijas mērīšanai tilpuma procentos sertificēta bezūdens etanola un dejonizēta ūdens maisījumiem. Pārbaužu rezultāti pierādīja, ka sensora kapacitātes vērtības ir stabili nosakāmas ar izšķirtspēju ne mazāku par ±0,005 pF. Sensora kapacitāšu vērtībām atbilstošā etanola tilpuma koncentrācijas atkārtojamu mērījumu izšķirtspēja visā mērīšanas diapazonā nebija mazāka par ±0,01%. Šajā darbā piedāvātajā jaunajā mērīšanas sistēmas struktūras risinājumā iekļautas divas tiešas signālu digitālās sintēzes mikrosistēmas, kas ar augstu precizitāti ģenerē mērīšanai nepieciešamās formas, fāzes un amplitūdas signālus ar 100,000 kHz frekvenci un tiek vadītas no mikrokontrolera. Mērīšanas sistēmā izmantota mērsignālu sinhronizētās detektēšanas tehnika. Izstrādātās sistēmas konigurācija, mērīšanas režīma uzstādījums un vadība automātiski praktiski izslēdz virkni mērīšanas signālu parazītisko fāzes nobīžu un fāzes uzstādījumu kļūdas. Izstrādātā mērīšanas sistēma ērti piemērojama vispārējai izmantošanai kapacitātes vai impedances mērierīcēs. Samērā nelielā prognozējamā mērīšanas sistēmas pašizmaksa un kompaktā, portatīvā konstrukcija, kā arī iepriekš minētās priekšrocības paplašina sistēmas lietojumu ne tikai etanola koncentrācijas mērīanai bioetanola ražošanas procesā, bet šī sistēma var tikt izmantota arī eļļas, benzīna un citu degvielu testēšanai, ķīmiskajā, farmācijas un pārtikas industrijā, mikrobioloģijā un medicīnā, lai noteiktu glikozes, antigēnu, antivielu, proteīna, DNS fragmentu un citu vielu saturu šķīdumos u.c.