Annotation Detail

Information
Associated Genes
PRNP
Associated Variants
PRNP p.Ala133Val (p.A133V) ( ENST00000379440.9, ENST00000424424.2, ENST00000430350.2, ENST00000457586.2 )
PRNP p.Ala133Val (p.A133V) ( ENST00000379440.9, ENST00000424424.2, ENST00000430350.2, ENST00000457586.2 )
Associated Disease
Gerstmann-Straussler-Scheinker syndrome
Source Database
ClinVar
Description
NM_000311.5(PRNP):c.398C>T (p.Ala133Val) AND Gerstmann-Straussler-Scheinker syndrome
ClinVar Allele ID
28453
ClinVar RefSeq Alternation Syntax
NM_000311.5:c.398C>T
ClinVar RefSeq Alternation Syntax
NM_001271561.3:c.*87C>T
ClinVar RefSeq Alternation Syntax
NM_001080122.3:c.398C>T
ClinVar RefSeq Alternation Syntax
NM_183079.4:c.398C>T
ClinVar RefSeq Alternation Syntax
NM_001080123.3:c.398C>T
ClinVar RefSeq Alternation Syntax
NM_001080121.3:c.398C>T
Clinical Significance Description
Pathogenic
Clinical Significance Last Update
2007-03-13
Clinical Significance Review Status
no assertion criteria provided
URL
https://www.ncbi.nlm.nih.gov/clinvar/RCV000014356
ClinVar Disease
Gerstmann-Straussler-Scheinker syndrome
Observed Origin Sample
germline
Pubmed
17353478
Drugs