Akademik Veri Yönetim Sistemi
SENDROM, cilt.15, sa.2, ss.97-107, 2003 (Scopus)
Transmissible spongiform encephalopathies (TSEs), cause by prions, are a group of rapidly progressive, invariably fatal, neurodegenerative diseases that affect both humans and animals. Most TSEs are characterized by a long incubation period and a neuropathologic feature of multifocal spongiform changes, astrogliosis, neuronal loss, and absence of inflammatory reaction. TSEs in humans include Creutzfeldt-Jakob disease (CJD), kuru, Gerstmann-Sträussler-Scheinker syndrome, fatal familial insomnia, and new variant CJD. Prions are proteinaceous infectious particles that lack nucleic acids. Prions are composed largely, if not entirely, of ah abnormal isoform of a normal cellular protein. Recently, TSEs attracted considerable public attention because of a 1996 report in the United Kingdom that bovine spongiform encephalopathy (BSE) may have spread to humans and caused a newly recognized variant form of CJD. BSE was first recognized in 1986 in Great Britain where a total of 181.883 cases were confirmed during 1986-December 2000. BSE, called "mad cow disease", is a progressive neurological disorder of cattle, that results from infection by an unconventional transmissible agent. The risk of infection for human by BSE prions is unclear. There is neither any treatment nor a vaccine to prevent the TSEs. In this article prions and characteristics of BSE and other prion diseases in human beings reviewed.