Abstract
Prion diseases are known as Transmissible Spongiform Encephalopathies (TSE). These are degenerative brain disorders characterized by tiny microscopic holes that give the brain 'spongy' appearance. The causative agent is proteinaceous infective particle called prion. Prion diseases affect a variety of mammals including humans. The disease is transmitted by contaminated food or feed containing prion protein. In animals the diseases caused by prions are Scrapie, Bovine Spongiform Encephalopathy (BSE), Transmissible Mink Encephalopathy (TME), Chronic Wasting Disease (CWD), Feline Spongiform Encephalopathy (FSE) and exotic Engulate Encephalopathy (EUE). Currently the only reliable test is histo-pathological examination of tissues. Control measures are surveillance, culling sick animals and banning specified risk materials. In India no case of BSE has been reported so far but the disease warrants constant monitoring and surveillance if once introduced or imported would be a herculean task to eradicate it.
Highlights
Prion diseases known as transmissible spongiform encephalopathies (TSE) are a group of rare degenerative brain disorders characterized by tiny microscopic holes that give the brain 'spongy' appearance
In animals the diseases caused by prions are, scrapie in sheep and goats, bovine spongiform encephalopathy (BSE) in cattle, transmissible mink encephalopathy (TME) in mink, chronic wasting disease (CWD) in white tailed deer, elk, mule deer and moose, feline spongiform encephalopathy in cats, exotic ungulate encephalopathy (EUE) in nyala and oryx
The results showed that PLL strongly inhibited abnormal prion protein (PrPsc) propagation in the cell-free, cell culture, and mouse models of prion disease
Summary
Prion diseases known as transmissible spongiform encephalopathies (TSE) are a group of rare degenerative brain disorders characterized by tiny microscopic holes that give the brain 'spongy' appearance. According to the prion theory, the agent of BSE is composed largely, but not entirely, of a selfreplicating protein, referred to as a prion. Another theory argues that the agent is virus-like and possesses nucleic acids which carry genetic information. It is known to be preventable through elimination of BSE contaminated feed and has been causally linked to vCJD in humans. This typical strain has not yet been identified in any U.S.A. born cattle. Reported French surveillance data support this theory that unlike typical BSE, atypical BSE appears to represent sporadic disease [9]
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