What is prion protein amyloidosis (APrP)?

Updated: May 09, 2019
  • Author: Robert O Holmes, Jr, DO; Chief Editor: Herbert S Diamond, MD  more...
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Answer

Answer

The precursor protein in APrP is a prion protein, which is a plasma membrane glycoprotein. The etiology is either infectious (ie, kuru) and transmissible spongiform encephalitis (TSE) or genetic (ie, Creutzfeldt-Jakob disease [CJD], Gerstmann-Sträussler-Scheinker [GSS] syndrome, fatal familial insomnia [FFI]). The infectious prion protein is a homologous protein encoded by a host chromosomal gene, which induces a conformational change in a native protease-sensitive protein, increasing the content of beta-pleated sheets. The accumulation of these beta-pleated sheets renders the protein protease-resistant and therefore amyloidogenic. [65] Patients with TSE, CJD, GSS, and FFI carry autosomal dominant amyloidogenic mutations in the prion protein gene; therefore, the amyloidosis forms even in the absence of an infectious trigger.

Similar infectious animal disorders include scrapie in sheep and goats and bovine spongiform encephalitis (ie, mad cow disease).


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