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

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

Gene information from NCBI Gene. Variant classifications from ClinVar.