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Lecture 9

BCH2011: Textbook summary - Lecture 9

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General Education Studies

LECTURE 9 Defects in Protein Provide the Molecular Basis for a Wide Range of Human Genetic Disorders: Despite the many processes that assist in protein folding, misfolding does occur. In fact, protein misfolding is a substantial problem in all cells, and a quarter or more of all polypeptides synthesized may be destroyed because they do not fold correctly. In some cases, the misfolding causes or contributes to the development of serious disease. The Prion Diseases: A misfolded brain protein seems to be the causative agent of several rare degenerative brain diseases in mammals. Perhaps the best known of these is mad cow disease, kuru, Creutzfeldt-Jakob in humans, Scrapie in sheep, and chronic wasting disease in deer and elk. These diseases are also referred to as spongiform encephalopathies because the diseased brain frequently becomes riddled with holes. The diseases are fatal. The infectious agent has been traced to a single protein, called prion protein (PrP). Prion protei
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