Background:
The protein encoded by this gene is a membrane glycosylphosphatidylinositol-anchored glycoprotein that tends to aggregate into rod-like structures. The encoded protein contains a highly unstable region of five tandem octapeptide repeats. This gene is found on chromosome 2; approximately 2 kbp upstream of a gene which encodes a biochemically and structurally similar protein to the one encoded by this gene. Mutations in the repeat region as well as elsewhere in this gene have been associated with Creutzfeldt-Jakob disease; fatal familial insomnia; Gerstmann-Straussler disease; Huntington disease-like 1; and kuru. An overlapping open reading frame has been found for this gene that encodes a smaller; structurally unrelated protein; AltPrp. Alternative splicing results in multiple transcript variants. [provided by RefSeq; Nov 214]