Annotation Detail

Information
Associated Genes
SMPD1
Associated Variants
SMPD1 p.Leu262ArgfsTer4 (p.L262Rfs*4) ( ENST00000342245.9, ENST00000527275.5 )
SMPD1 p.Leu262ArgfsTer4 (p.L262Rfs*4) ( ENST00000342245.9, ENST00000527275.5 )
Associated Disease
Niemann-Pick disease, type A
Source Database
ClinVar
Description
NM_000543.5(SMPD1):c.785_807del (p.Leu262fs) AND Niemann-Pick disease, type A
ClinVar Allele ID
192247
ClinVar RefSeq Alternation Syntax
NM_000543.5:c.785_807del
ClinVar RefSeq Alternation Syntax
NR_027400.3:n.910_932del
ClinVar RefSeq Alternation Syntax
NM_001007593.3:c.782_804del
ClinVar RefSeq Alternation Syntax
NM_001318087.2:c.785_807del
ClinVar RefSeq Alternation Syntax
NM_001318088.2:c.-177_-155del
ClinVar RefSeq Alternation Syntax
NM_001365135.2:c.785_807del
Clinical Significance Description
Pathogenic
Clinical Significance Last Update
2023-08-17
Clinical Significance Review Status
criteria provided, single submitter
URL
https://www.ncbi.nlm.nih.gov/clinvar/RCV000984222
ClinVar Disease
Niemann-Pick disease, type A
Observed Origin Sample
germline
Observed Origin Sample
unknown
Drugs