PRNP

(redirected from PrP27-30)

PRNP

A gene on chromosome 20p13 that encodes a membrane glycosyl-phosphatidylinositol-anchored glycoprotein, which aggregates into rod-like structures and contains a highly unstable region of five tandem octapeptide repeats. The exact function of PrP is unknown.

Molecular pathology
PRNP mutations are linked to Creutzfeldt-Jakob disease, fatal familial insomnia, Gerstmann-Straussler disease, Huntington-like disease 1 and kuru.
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The misfolded prion protein comprises an N terminal protease-sensitive part followed by a region with variable protease sensitivity and a C-terminal protease-resistant core referred to as [PrP.sup.res] or PrP27-30 (Figure 1).