Prion Diseases
Microglia in prion diseases
... in prion disease, at least partly by engulfing and degrading prions; however, phagocy- tosis and the clearance of prions are inefficient, and animals eventually die of the disease ...of prion-infected mice ...
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Familial human prion diseases associated with prion protein mutations Y226X and G131V are transmissible to transgenic mice expressing human prion protein
... familial prion diseases are associated with mutations at 34 different prion protein (PrP) amino acid ...of prion infectivity from a patient expressing truncated human PrP may have implications ...
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Amylotrophic Lateral Sclerosis Like Motor Impairment in Prion Diseases
... of Prion diseases, motor impairment is the commonest at the different stages of disease ...neuron diseases where both or either of Upper motor neuron (UMN) or lower motor neuron (LMN) that arise from ...
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Proteinase-activated receptor 2 and disease biomarkers in cerebrospinal fluid in cases with autopsy-confirmed prion diseases and other neurodegenerative diseases
... In the CNS, PARs are expressed on neurons, glia, ependymal cells and endothelial cells and modulate both neuroprotective and neurotoxic effects [2]. In a recent study using a murine prion disease model, PAR-2 ...
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Schwengler, Franziska (2005): Prion Diseases: A Genetic Perspective. Dissertation, LMU München: Tierärztliche Fakultät
... of prion diseases to cross species barriers was highlighted by the occurrence of a new variant of CJD (vCJD) in the UK (Will et ...human prion diseases like sporadic CJD with regard to plaque ...
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Genomic and post-genomic analyses of human prion diseases
... different prion diseases depending on whether the mutation co-segregates with methionine (FFI) or valine (genetic CJD) in the polymorphic codon ...genetic prion diseases remains to be ...
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Progress and problems in the biology, diagnostics, and therapeutics of prion diseases
... Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are invariably fatal neurodegenerative disorders affecting a broad spectrum of host species and arise via genetic, infectious, ...
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PrPScaccumulation in neuronal plasma membranes links Notch-1 activation to dendritic degeneration in prion diseases
... in prion diseases, before the defining neuro- pathological change of vacuolar degeneration of gray ...of prion disease and it precedes nerve cell death, which is a late morphological ...in ...
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Filamentous white matter prion protein deposition is a distinctive feature of multiple inherited prion diseases
... of prion diseases including vCJD [6] and some cases of 129MM sCJD [7] and in the cerebellum of 129VV patients ...inherited prion diseases, white matter plaques have been described before, and ...
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Absence of CD14 Delays Progression of Prion Diseases Accompanied by Increased Microglial Activation
... of prion diseases. To an- alyze the host reaction in prion infection in the brain, particularly during the early stages after intracerebral inoculation of prions, we compared the gene expression of ...
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Molecular dynamic simulation studies of Q212H, V203G and N173K mutations in prion diseases
... Currently, Prion diseases in humans are grouped based on whether they are sporadic, inherited, or ...cases prion disease are sporadic; meaning, they develop spontaneously with no known ...hand, ...
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Orally Administered Amyloidophilic Compound Is Effective in Prolonging the Incubation Periods of Animals Cerebrally Infected with Prion Diseases in a Prion Strain-Dependent Manner
... with prion disease ...or prion diseases, are effective as antiprion chemicals (15, ...be prion strain dependent, but this was not fully evaluated because of the limited availability of the ...
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Low activity of complement in the cerebrospinal fluid of the patients with various prion diseases
... Fig. 3 Detection of individual CSF levels of the total complement activity among a sampling of human prion diseases using indirect ELISAs which indicate 50 % of complement hemolytic activity. The solid line ...
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Combined Diffusion Imaging and MR Spectroscopy in the Diagnosis of Human Prion Diseases
... hand, be performed simultaneously with conventional imaging and can give information on the ongoing pathologic processes: Neuronal loss or stress will lead to a decrease in NAA, while gliosis will induce an increase in ...
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Interaction of prion protein with acetylcholinesterase: potential pathobiological implications in prion diseases
... -enzymatic function of AChE in PrP misfolding and aggregation could be relevant in prion diseases. Indeed, reduced AChE activity in lumbar CSF samples and altered AChE glycosylation in the brain and CSF ...
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Epidemiological characteristics of human prion diseases
... human prion diseases, sCJD, occurs equally in both sexes with a peak age of onset between 60 and 69 years ...with prion diseases (most of them with the sCJD subtype), in which the mean disease ...
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Identification and structural analysis of C-terminally truncated collapsin response mediator protein-2 in a murine model of prion diseases
... scrapie prion (Obihiro I strain), we found that the amount of the full-length form of collapsin response mediator protein-2 (CRMP-2; 61 kDa) decreased in the late stages of the disease, while the amount of its ...
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An overview of animal prion diseases
... other prion diseases, clinico- pathological phenotypes of scrapie vary according to the prion strain and animals ’ genetic ...Multiple prion strains may exist in a single scrapie isolate and a ...
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An overview of human prion diseases
... Prion diseases are transmissible protein misfolding dis- orders in which misfolding of a host-encoded prion pro- tein (PrP) ...cellular prion pro- tein designated as PrP C and a pathogenic ...
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Molecular Pathogenesis of Prion Diseases
... inherited prion diseases and mutations in the PRNP ...sporadic prion diseases, the large majority of which occur in homozygous individuals (Collinge et ...
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