TY - JOUR T1 - An overview of human prion diseases. AU - Imran,Muhammad, AU - Mahmood,Saqib, Y1 - 2011/12/24/ PY - 2011/08/04/received PY - 2011/12/24/accepted PY - 2011/12/27/entrez PY - 2011/12/27/pubmed PY - 2012/6/13/medline SP - 559 EP - 559 JF - Virology journal JO - Virol J VL - 8 N2 - Prion diseases are transmissible, progressive and invariably fatal neurodegenerative conditions associated with misfolding and aggregation of a host-encoded cellular prion protein, PrP(C). They have occurred in a wide range of mammalian species including human. Human prion diseases can arise sporadically, be hereditary or be acquired. Sporadic human prion diseases include Cruetzfeldt-Jacob disease (CJD), fatal insomnia and variably protease-sensitive prionopathy. Genetic or familial prion diseases are caused by autosomal dominantly inherited mutations in the gene encoding for PrP(C) and include familial or genetic CJD, fatal familial insomnia and Gerstmann-Sträussler-Scheinker syndrome. Acquired human prion diseases account for only 5% of cases of human prion disease. They include kuru, iatrogenic CJD and a new variant form of CJD that was transmitted to humans from affected cattle via meat consumption especially brain. This review presents information on the epidemiology, etiology, clinical assessment, neuropathology and public health concerns of human prion diseases. The role of the PrP encoding gene (PRNP) in conferring susceptibility to human prion diseases is also discussed. SN - 1743-422X UR - https://www.unboundmedicine.com/medline/citation/22196171/An_overview_of_human_prion_diseases_ L2 - https://virologyj.biomedcentral.com/articles/10.1186/1743-422X-8-559 DB - PRIME DP - Unbound Medicine ER -