Transmissible spongiform encephalopathies (TSE), or prion diseases, are a family of inevitably fatal neurodegenerative disorders affecting a variety of mammalian species.These diseases include scrapie in sheep and goats; bovine spongiform encephalopathy (BSE, "mad cow" disease) in cattle; chronic wasting disease (CWD) in North American deer, elk and moose; transmissible mink encephalopathy; and Creutzfeldt-Jakob disease (CJD, sporadic, familial and variant forms) and kuru in humans.These diseases are characterized by long incubation periods, spongiform degeneration of the brain and accumulation of an abnormally folded isoform of the prion protein, designated PrP Sc , in brain tissue (Prusiner 1998).
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Schramm et al. (2006) studied this question.
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