Prion DiseasesJohn Collinge (MD.), Mark S. Palmer The number of neurological conditions associated with the mutant "prion" proteins continues to grow. The list includes BSE and scrapie, which affect cattle and sheep respectively, and Creutzfeldt-Jacob Disease, which affects humans. This is an area of intense interest to neuroscientists, veterinary scientists, and clinicians. It has also attracted significant media attention with the recent outbreak of mad cow disease in Britain because of the potential risks to humans. This book brings together leading researchers in the field, including S. Prusiner--the researcher who first suggested that the cause of prion diseases is a protein rather than a virus. The volume represents the most up-to-date and authoritative summary available. It describes the basic structural properties of the prion protein, covers the cell biology and transgenic models of prion disease, and discusses the pathology of the diseases affecting animals and humans. The authors also provide an historical perspective and look into the future implications of current research. |
Contents
List of contributors | 1 |
Human prion diseases | 18 |
Pathology of prion diseases | 57 |
Copyright | |
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Common terms and phrases
abnormal allele Alpers Alzheimer's disease amino acid amyloid plaques animals ataxia bovine spongiform encephalopathy brain cattle cells cellular cerebellar clinical signs codon Collinge Creutzfeldt-Jakob disease DeArmond dementia detected developed diagnosis disorders Fatal familial insomnia fibrils Gajdusek genetic genotype goats Goldfarb Hadlow Hsiao human prion diseases iatrogenic immunocytochemistry incubation period infection inherited prion disease inoculation isoform Journal Kimberlin Kitamoto kuru Lancet lesions membrane molecular molecules mouse mutation Neurology neuronal loss neuropathology normal occur onset pathology patients peptide phenotype prion diseases prion protein prion protein gene PRNP protease-resistant PrP amyloid plaques PrP gene PrPC PrPsc Prusiner region reported Scott scrapie scrapie agent scrapie prion scrapie strains sequence SHaPrP sheep species spongiform change sporadic CJD studies susceptibility synaptic Syrian hamsters Taraboulos Tateishi Tg(MH2M PrP)92 mice tion tissues transgenic transmissible mink encephalopathy transmissible spongiform encephalopathies vacuolation Veterinary Record Virology Westaway Wilesmith