Annotation Detail

Information
Associated Genes
SMPD1
Associated Variants
SMPD1 p.Ser192AlafsTer65 (p.S192Afs*65) ( ENST00000527275.5, ENST00000342245.9 )
SMPD1 p.Ser192AlafsTer65 (p.S192Afs*65) ( ENST00000342245.9, ENST00000527275.5 )
Associated Disease
Niemann-Pick disease, type B
Source Database
ClinVar
Description
NM_000543.5(SMPD1):c.573del (p.Ser192fs) AND Niemann-Pick disease, type B
ClinVar Allele ID
178086
ClinVar RefSeq Alternation Syntax
NM_000543.5:c.573del
ClinVar RefSeq Alternation Syntax
NM_001318087.2:c.573del
ClinVar RefSeq Alternation Syntax
NM_001318088.2:c.-389del
ClinVar RefSeq Alternation Syntax
NM_001007593.3:c.570del
ClinVar RefSeq Alternation Syntax
NM_001365135.2:c.573del
ClinVar RefSeq Alternation Syntax
NR_027400.3:n.698del
Clinical Significance Description
Pathogenic
Clinical Significance Last Update
2018-01-09
Clinical Significance Review Status
no assertion criteria provided
URL
https://www.ncbi.nlm.nih.gov/clinvar/RCV000983993
ClinVar Disease
Niemann-Pick disease, type B
Observed Origin Sample
unknown
Drugs