Characterization of Truncated Forms of Abnormal Prion Protein in Creutzfeldt-Jakob Disease
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چکیده
منابع مشابه
LETTERS Irregular presence of abnormal prion protein in appendix in variant Creutzfeldt-Jakob disease
We have investigated the presence of disease related prion protein (PrP) in appendix samples obtained at necropsy from four neuropathologically confirmed cases of variant Creutzfeldt-Jakob disease (vCJD). PrP was detected in only one vCJD appendix, at a level lower than found in a diagnostic tonsil biopsy sample obtained from the same patient. The single PrP positive appendix, but not the other...
متن کاملIrregular presence of abnormal prion protein in appendix in variant Creutzfeldt-Jakob disease.
We have investigated the presence of disease related prion protein (PrP) in appendix samples obtained at necropsy from four neuropathologically confirmed cases of variant Creutzfeldt-Jakob disease (vCJD). PrP was detected in only one vCJD appendix, at a level lower than found in a diagnostic tonsil biopsy sample obtained from the same patient. The single PrP positive appendix, but not the other...
متن کاملDiagnosing Sporadic Creutzfeldt-Jakob Disease by the Detection of Abnormal Prion Protein in Patient Urine.
Importance Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder associated with the accumulation of infectious abnormal prion protein through a mechanism of templated misfolding. A recent report has described the detection of abnormal prion protein in the urine of patients with variant CJD (vCJD) using protein misfolding by cyclic amplification, which was apparently absent in t...
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 کاملNovel 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...
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ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 2008
ISSN: 0021-9258
DOI: 10.1074/jbc.m801877200