Variant CJD

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Variant CJD

Abbreviations: BSE, bovine spongiform encephalopathy; CWD, chronic wasting disease; GSS, Gerstman–Str€aussler–Scheinker disease; M, methionine; PPS, pentosan polysulphate; PrP, protease-resistant prion protein; PrP, abnormal prion protein; QuIC, quaking-induced conversion; TSE, transmissible spongiform encephalopathy; V, valine; vCJD, variant Creutzfeldt–Jakob disease; VPSPr, variably protease-...

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The molecular epidemiology of variant CJD.

The emergence of the novel prion diseases bovine spongiform encephalopathy (BSE) and, subsequently, variant Creutzfeldt-Jakob disease (vCJD) in epidemic forms has attracted much scientific attention. The oral transmission of these disorders, the causative relationship of vCJD to BSE and the resistance of the transmissible agents in both disorders to conventional forms of decontamination has cau...

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Public health risks from subclinical variant CJD

Variant Creutzfeldt-Jakob disease (vCJD) is a zoonotic prion disease thought to have been transmitted to humans through the consumption of food products contaminated with bovine spongiform encephalopathy (BSE) in the 1980s and/or early 1990s. As with all prion disorders, it is a fatal neurodegenerative disease arising from conversion of the normal cellular form of the prion protein PrP, encoded...

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Human Prion Protein V129 Prevents Expression of Variant CJD Phenotype

Materials and Methods Transgenic mice. Transgenic mice homozygous for a human PrP 129V transgene array and murine PrP null alleles (Prnp) designated Tg(HuPrP129V Prnp)-152 mice (129VV Tg152 mice) and transgenic mice homozygous for a human PrP 129M transgene array and murine PrP null alleles (Prnp) designated Tg(HuPrP129M Prnp)-35 mice (129MM Tg35 mice) have been described previously (S1-S4). In...

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CLINICAL ETHICS Ethical considerations in presymptomatic testing for variant CJD

Variant Creutzfeldt–Jakob disease (vCJD) is a fatal, transmissible, neurodegenerative disorder for which there is currently no effective treatment. vCJD arose from the zoonotic spread of bovine spongiform encephalopathy. There is now compelling evidence for human to human transmission through blood transfusions from presymptomatic carriers and experts are warning that the real epidemic may be y...

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ژورنال

عنوان ژورنال: Prion

سال: 2014

ISSN: 1933-6896,1933-690X

DOI: 10.4161/pri.29237