Novel Prion Protein Conformation and Glycotype in Creutzfeldt-Jakob Disease
نویسندگان
چکیده
منابع مشابه
Novel prion protein conformation and glycotype in Creutzfeldt-Jakob disease.
OBJECTIVE To describe a novel molecular and pathological phenotype of Creutzfeldt-Jakob disease. Patient A 69-year-old woman with behavioral and personality changes followed by rapidly evolving dementia. RESULTS Postmortem examination of the brain showed intracellular prion protein deposition and axonal swellings filled with amyloid fibrils. Biochemical analysis of the pathological prion prot...
متن کاملpH-dependent prion protein conformation in classical Creutzfeldt-Jakob disease.
In transmissible spongiform encephalopathies, the cellular prion protein (PrP(C)) undergoes a conformational change from a prevailing alpha-helical structure to a beta-sheet-rich, protease-resistant isoform, termed PrP(Sc). PrP(C) has two characteristics: a high affinity for Cu(2+) and a strong pH-dependent conformation. Lines of evidence indicate that PrP(Sc) conformation is dependent on coppe...
متن کاملExtraneural pathologic prion protein in sporadic Creutzfeldt-Jakob disease.
BACKGROUND In patients with sporadic Creutzfeldt-Jakob disease, pathologic disease-associated prion protein (PrPSc) has been identified only in the central nervous system and olfactory-nerve tissue. Understanding the distribution of PrPSc in Creutzfeldt-Jakob disease is important for classification and diagnosis and perhaps even for prevention. METHODS We used a highly sensitive method of det...
متن کاملNovel prion protein gene mutation in an octogenarian with Creutzfeldt-Jakob disease.
BACKGROUND The transmissible spongiform encephalopathies constitute a fascinating and biologically unique group of invariably fatal neurodegenerative disorders that affect both animals and humans. Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker syndrome, and fatal familial insomnia represent the more common human phenotypes. Excluding the small number of iatrogenically transmitt...
متن کاملPrion infections in Creutzfeldt-Jakob disease and its variants.
Prions (PrP(Sc)) are proteinaceous infectious particles that occur as sporadic (85 percent), infectious (iatrogenic) (5 percent) or hereditary (10 percent) diseases in humans and animals. These unique infectious agents produce a spongiform change in the central nervous system without any inflammation, inclusion bodies or apparent antibody response. A helper (X) protein and genetic predispositio...
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ژورنال
عنوان ژورنال: Archives of Neurology
سال: 2007
ISSN: 0003-9942
DOI: 10.1001/archneur.64.4.595