PRNP

prion protein (Kanno blood group)

Summary

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 20, approximately 20 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 2014]

Known Variants150 total

rsidPosition (GRCh37)AllelesClassClinVar
rs275627120:4,665,262A/T——
rs5763365620:4,666,109T/Gupstream gene variant—
rs76547180720:4,666,859T/A—uncertain significance
rs1304032720:4,666,924C/G—benign
rs88605673920:4,666,958G/A—uncertain significance
rs88605674020:4,666,963C/G—uncertain significance
rs88605674120:4,667,147C/G—uncertain significance
rs88605674220:4,667,148G/T—uncertain significance
rs53744934020:4,667,151C/T—benign
rs610751620:4,677,092G/Aintron variant—
rs7823663120:4,679,836G/A—benign
rs74822783720:4,679,871C/T—conflicting classifications of pathogenicity
rs140525798220:4,679,872G/A—likely benign
rs36815457920:4,679,916G/C—uncertain significance
rs54811656420:4,679,920C/T—likely benign
rs149041341320:4,679,923G/A—likely benign
rs14693973220:4,679,940G/A—uncertain significance
rs1153875520:4,679,942C/G—uncertain significance
rs75696932020:4,679,944G/A—likely benign
rs98926479920:4,679,952G/A—uncertain significance
rs251437191820:4,679,954G/A—uncertain significance
rs131385568620:4,679,976G/A—uncertain significance
rs74701999020:4,679,982C/T—conflicting classifications of pathogenicity
rs77120686820:4,679,983G/A—likely benign
rs20210237820:4,679,986G/A—benign
rs251437223020:4,680,004C/T—likely benign
rs94513646720:4,680,009G/A—conflicting classifications of pathogenicity
rs77618895020:4,680,025C/T—likely benign
rs76352438020:4,680,026G/A—likely benign
rs138672070320:4,680,046T/C—uncertain significance
rs75006967920:4,680,064G/A—uncertain significance
rs53249311420:4,680,070T/C—benign
rs124756336220:4,680,072A/T—uncertain significance
rs120955353520:4,680,080G/C—uncertain significance
rs77692217320:4,680,091G/A—likely benign
rs11263743720:4,680,094C/T—likely benign
rs6264336420:4,680,112A/G—benign
rs6263768620:4,680,118T/C—benign
rs251437322420:4,680,144G/A—uncertain significance
rs192241894520:4,680,152C/G—uncertain significance
rs5636294220:4,680,156G/A—uncertain significance
rs7431540120:4,680,171C/Tmissense variantpathogenic
rs37042610020:4,680,172G/A—likely benign
rs7431541420:4,680,179C/Amissense variantpathogenic
rs1153875820:4,680,180C/Tmissense variantpathogenic
rs77289909720:4,680,199C/T—likely benign
rs7431540220:4,680,216C/Tmissense variantpathogenic
rs812421420:4,680,217G/A—benign
rs75740925820:4,680,232G/T—likely benign
rs20142399020:4,680,238C/G—likely benign
rs26760698020:4,680,246G/Tmissense variantprotective
rs179999020:4,680,251A/Gmissense variantpathogenic
rs7431541020:4,680,258G/Tmissense variantpathogenic
rs77273588920:4,680,259A/G—likely benign
rs7431541520:4,680,264C/Tmissense variantpathogenic
rs192243494820:4,680,268G/C—uncertain significance
rs14555509620:4,680,273G/C—uncertain significance
rs14631584620:4,680,274G/T—uncertain significance
rs97780175520:4,680,280C/G—uncertain significance
rs155578205620:4,680,281A/G—uncertain significance
rs76492085420:4,680,289C/T—likely benign
rs15035164420:4,680,290G/A—likely benign
rs135817452820:4,680,298C/G—uncertain significance
rs8035671020:4,680,301T/Gstop gainedpathogenic
rs143696806620:4,680,307C/T—likely benign
rs18134829920:4,680,309G/A—likely pathogenic
rs75541730120:4,680,317C/T—uncertain significance
rs77911326820:4,680,318G/T—uncertain significance
rs14430226720:4,680,328G/A—uncertain significance
rs132347820620:4,680,333G/A—uncertain significance
rs251437400520:4,680,339C/T—uncertain significance
rs8035671120:4,680,344C/Tstop gainedpathogenic
rs192244717320:4,680,346A/G—likely benign
rs74652697720:4,680,349G/A—likely benign
rs92154280620:4,680,353T/C—uncertain significance
rs155578210120:4,680,355C/G—pathogenic
rs77042274920:4,680,359C/T—uncertain significance
rs141961856020:4,680,363T/C—uncertain significance
rs74548063920:4,680,364G/A—uncertain significance
rs105734322420:4,680,371T/C—uncertain significance
rs76944798620:4,680,373C/T—likely benign
rs1699001820:4,680,378A/Gmissense variantuncertain significance
rs14972657920:4,680,385C/T—likely benign
rs20029421420:4,680,397C/T—likely benign
rs7431540320:4,680,398G/Amissense variantpathogenic
rs75518366220:4,680,403C/T—likely benign
rs7431540820:4,680,404G/Amissense variantpathogenic
rs75301500920:4,680,412C/G—uncertain significance
rs7431541120:4,680,413A/Gmissense variantpathogenic
rs7431541320:4,680,426A/Gmissense variantpathogenic
rs37287879120:4,680,429C/G—likely pathogenic
rs20144774220:4,680,430G/A—likely benign
rs117846684820:4,680,431G/A—uncertain significance
rs116371735820:4,680,457C/T—likely benign
rs7431540520:4,680,459T/Cmissense variantpathogenic
rs2893338520:4,680,464G/Amissense variantpathogenic
rs14555658920:4,680,469C/T—likely benign
rs37194826920:4,680,472C/T—conflicting classifications of pathogenicity
rs212223038820:4,680,486A/G—uncertain significance
rs5582623620:4,680,488C/T—uncertain significance

Showing 100 of 150 variants. Use the SNP search for the full list.

Gene information from NCBI Gene. Variant classifications from ClinVar.