Prion-like protein doppel (PRND)

Prion-like protein doppel (PRND) is a membrane glycosylphosphatidylinositol (GPI)-anchored glycoprotein with significant structural similarity to the cellular prion protein (PrPC), sharing about 25% sequence identity in key regions[1][3]. The PRND gene is located on chromosome 20 in humans, close to the prion protein gene (PRNP)[3][5]. Unlike PrPC, PRND (Doppel) is predominantly expressed in the testis and is required for normal acrosome function and male fertility, with much lower expression in the adult central nervous system[1][3][5]. It is not involved in the propagation of misfolded prion protein associated with transmissible spongiform encephalopathies, but its role in neurodegenerative disease remains under investigation, as animal studies have linked doppel overexpression in the brain to neurodegeneration[1][5]. Mutations in PRND may be associated with neurodegenerative disease risk, but evidence in humans is contradictory and does not establish a clear causal link[1][5]. No drugs are currently known to target PRND directly, and it is not considered a therapeutic target at this time.

Other names

Prion protein 2 (dublet)Doppel proteinDPLDOPPELPrPLPUNQ1830/PRO3443dJ1068H6.4downstream prion protein-like geneprion gene complex, downstreamprion protein 2