Human prion diseases

R G Will

in Oxford Textbook of Medicine

Published on behalf of Oxford University Press

Published in print May 2010 | ISBN: 9780199204854
Published online November 2012 | e-ISBN: 9780199570973 | DOI:

Series: Oxford Textbooks

Human prion diseases

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Prion protein (for proteinacious infectious particle) is a membrane-associated glycoprotein present in all mammalian species. Its normal function is unknown, but in prion diseases (also known as transmissible spongiform encephalopathies) a post-translationally modified form of the protein, partially resistant to protease digestion, is deposited in the brain and associated—after long incubation periods—with neuronal dysfunction and death....

Chapter.  6930 words.  Illustrated.

Subjects: Neurology

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