Molecular Pathology of Human Prion Diseases
نویسندگان
چکیده
منابع مشابه
Molecular Pathology of Human Prion Diseases
Prion diseases are fatal neurodegenerative conditions in humans and animals. In this review, we summarize the molecular background of phenotypic variability, relation of prion protein (PrP) to other proteins associated with neurodegenerative diseases, and pathogenesis of neuronal vulnerability. PrP exists in different forms that may be present in both diseased and non-diseased brain, however, a...
متن کاملThe molecular pathology of prion diseases.
15 Abstract Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of invariably fatal neurodegenerative disorders. Uniquely, they may present as sporadic, inherited, or infectious forms, all of which involve conversion of the normal cellular prion protein (PrP) into a pathogenic likeness of itself (PrP). Formation of neurotoxic PrP and/or loss of the normal function o...
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 کاملVisual pathology in animal prion diseases.
Prion diseases, also known as the transmissible spongiform encephalopathies (TSEs), are a group of slowly developing neurodegenerations occurring in human and animals. Prion diseases can be transferred between animals, humans, from humans to animals, and from animals to humans. As a result, the central nervous system is attacked, resulting in microglia activation, astrocytosis, prion plaque dep...
متن کاملMolecular Pathogenesis of Prion Diseases
In humans, prion diseases occur with unique aetiology as sporadic, genetic or infectious disorders. Sporadic cases of prion diseases, which account for the majority of casualties (up to 85% of all cases), are of unknown origin; the genetic forms are less frequent (up to 15%), while the infectious cases are extremely rare with an incidence of less than 1% (Prusiner, 2001). Creutzfeldt-Jakob dise...
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ژورنال
عنوان ژورنال: International Journal of Molecular Sciences
سال: 2009
ISSN: 1422-0067
DOI: 10.3390/ijms10030976