Cellular Phenotyping of Secretory and Nuclear Prion Proteins Associated with Inherited Prion Diseases
نویسندگان
چکیده
منابع مشابه
Cellular biology of prion diseases.
Prion diseases are fatal neurodegenerative disorders of humans and animals that are important because of their impact on public health and because they exemplify a novel mechanism of infectivity and biological information transfer. These diseases are caused by conformational conversion of a normal host glycoprotein (PrPC) into an infectious isoform (PrPSc) that is devoid of nucleic acid. This r...
متن کاملCellular pathogenesis in prion diseases.
Prion diseases are characterised by neuronal loss, vacuolation (spongiosis), reactive astrocytosis, microgliosis and in most cases by the accumulation in the central nervous system of the abnormal prion protein, named PrP(Sc). In this review on the "cellular pathogenesis in prion diseases", we have chosen to highlight the main mechanisms underlying the impact of PrP(C)/PrP(Sc) on neurons: the n...
متن کاملFilamentous white matter prion protein deposition is a distinctive feature of multiple inherited prion diseases
BACKGROUND Sporadic, inherited and acquired prion diseases show distinct histological patterns of abnormal prion protein (PrP) deposits. Many of the inherited prion diseases show striking histological patterns, which often associate with specific mutations. Most reports have focused on the pattern of PrP deposition in the cortical or cerebellar grey matter. RESULTS We observed that the subcor...
متن کاملPrion and Prion-like Diseases in Humans
Introduction. Genetic prion diseases, such as Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker disease (GSS), Fatal Familial Insomnia (FFI), are associated with the mutations of prion (PRNP) gene. More than 30 pathogenic PRNP mutations were identified. Since Alzheimer disease (AD) and prion diseases have many similarities in the neuropathological and physiological symptoms, we te...
متن کاملPrion Diseases
Stanley Prusiner introduced the term prion (proteinaceous infectious particle) and proposed that the infectious agent in prion diseases is composed mainly or entirely of an abnormal conformation of an otherwise normal host-encoded glycoprotein called the prion protein (PrP)1. This hypothesis was initially greeted with great skepticism in the scientific community but then later was widely acknow...
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ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 2002
ISSN: 0021-9258
DOI: 10.1074/jbc.m110197200