Vaccines against transmissible spongiform Encephalopathies: An urgent need?

Steffen Bade and Andreas Frey

volume 4 | issue 1

january/february 2008
Pages: 79 - 81

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Transmissible spongiform encephalopathies (TSE) are fatal neurodegenerative disorders which can be acquired by humans and certain animals, predominantly ruminants. The disease is caused by a non-classical infectious agent, the so-called prions. Prions are proteinaceous agglomerates of remarkable biochemical stability which consist mainly, if not entirely, of misfolded isoforms (PrPSc) of the cellular prion protein (PrPc)1. Once an individual has been infected, the disease spreads in a cascadic mechanism in which prions replicate and accumulate throughout organs of the lymphoreticular system by catalyzing the misfolding of PrPc into PrPSc. In the terminal state of the affliction the PrPc-rich neural tissues become affected2 which leads to severe neurological symptoms such as ataxia and dementia. So far, TSE are lethal diseases with no cure in reach.

Authors

Steffen Bade

Research Center Borstel, Leibniz-Center for Medicine and Biosciences, Borstel, Germany

Andreas Frey