PRNP

(redirected from major prion protein)

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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In a tour de force of molecular biology, Prusiner's group, along with researchers led by Charles Weissmann at the University of Zurich, and by Leroy Hood at Caltech in Pasadena, located the gene that encodes the major prion protein.