Species barriers in prion diseases — brief review

作者: R. -A. Moore , I. Vorberg , S. -A. Priola1

DOI: 10.1007/3-211-29981-5_15

关键词:

摘要: Transmissible spongiform encephalopathies (TSEs or prion diseases) are neurological disorders associated with the aggregation of a pathologic isoform host-encoded protein, termed protein (PrP). The PrP, PrP(Sc), is major constituent infectious agent. TSE diseases characterized by neurodegenerative failure and inevitable morbidity. Bovine encephalopathy (BSE) has been transmitted from cattle to humans cause new variant Creutzfeldt-Jakob syndrome. potential for chronic wasting disease similarly cross species barrier cervids considered unlikely but possible. Thus, understanding how agents overcome resistance transmission between crucial if we prevent future epidemics. usually can be abrogated varying degrees in laboratory animals. Studies done transgenic animals, tissue culture, cell-free assays established PrP as being necessary pathogenesis illustrated that certain amino acid residues more influential than others conferring agent transmission. essence what constitutes agent's compatibility thought orchestrated complex interplay contributions its primary sequence, glycoform patterns, three-dimensional structure.

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