Annotation Detail

Information
Associated Genes
SMPD1
Associated Variants
SMPD1 p.Arg476Trp (p.R476W) ( ENST00000527275.5, ENST00000342245.9 )
SMPD1 p.Arg476Trp (p.R476W) ( ENST00000342245.9, ENST00000527275.5 )
Associated Disease
Niemann-Pick disease, type A
Source Database
ClinVar
Description
NM_000543.5(SMPD1):c.1426C>T (p.Arg476Trp) AND Niemann-Pick disease, type A
ClinVar Allele ID
99222
ClinVar RefSeq Alternation Syntax
NM_001007593.3:c.1423C>T
ClinVar RefSeq Alternation Syntax
NR_027400.3:n.1379C>T
ClinVar RefSeq Alternation Syntax
NM_001318087.2:c.1426C>T
ClinVar RefSeq Alternation Syntax
NM_001318088.2:c.505C>T
ClinVar RefSeq Alternation Syntax
NM_000543.5:c.1426C>T
ClinVar RefSeq Alternation Syntax
NM_001365135.2:c.1294C>T
ClinVar RefSeq Alternation Syntax
NR_134502.2:n.898C>T
Clinical Significance Description
Pathogenic/Likely pathogenic
Clinical Significance Last Update
2014-06-11
Clinical Significance Review Status
criteria provided, multiple submitters, no conflicts
URL
https://www.ncbi.nlm.nih.gov/clinvar/RCV000169170
ClinVar Disease
Niemann-Pick disease, type A
Observed Origin Sample
germline
Observed Origin Sample
unknown
Drugs