منابع مشابه
Therapies for human prion diseases.
The pathological foundation of human prion diseases is a result of the conversion of the physiological form of prion protein (PrP(c)) to the pathological protease resistance form PrP(res). Most patients with prion disease have unknown reasons for this conversion and the subsequent development of a devastating neurodegenerative disorder. The conversion of PrP(c) to PrP(res), with resultant propa...
متن کاملHuman prion diseases: molecular and clinical aspects.
Compared with that of other human pathogens, the proposed replicative cycle of prions is disarmingly simple. It encompasses misfolding of a single protein, the cellular prion protein (PrPC), into a disease-associated form called PrPSc. This is followed by PrPSc aggregation and possibly fragmentation of aggregates, which may augment the number of replicative units. Although there is no formal pr...
متن کاملEpidemiological characteristics of human prion diseases
Human prion diseases are a group of transmissible, progressive, and invariably fatal neurodegenerative disorders, which include Kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome, and fatal familial insomnia. Human prion diseases affect approximately 1-2 persons per million worldwide annually, occurring in sporadic, inherited, and acquired forms. These diseases have ...
متن کاملHuman prion diseases: progress in clinical trials
The progress made in understanding disease pathology and phenomenology in prion disorders and recent advances in diagnostic techniques might encourage researchers now to consider therapeutic trials in patients with Creutzfeldt–Jakob disease. Although attempts have been made in the past (Trevitt and Collinge, 2006; Stewart et al., 2008), the drugs tested involved a variety of compounds that belo...
متن کاملHuman Prion Diseases in the United States
BACKGROUND Prion diseases are a family of rare, progressive, neurodegenerative disorders that affect humans and animals. The most common form of human prion disease, Creutzfeldt-Jakob disease (CJD), occurs worldwide. Variant CJD (vCJD), a recently emerged human prion disease, is a zoonotic foodborne disorder that occurs almost exclusively in countries with outbreaks of bovine spongiform encepha...
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ژورنال
عنوان ژورنال: Journal of the Korean Medical Association
سال: 2008
ISSN: 1975-8456
DOI: 10.5124/jkma.2008.51.12.1125