GenTIGSA Gene Database on Rare Genetic Disorders

Variants in ClinVar from 539 of 916 Rare Genetic Disorders of GenTIGS

Niemann-Pick disease, type B

An  Autosomal recessive  mode(s) within the Metabolic disorders/Lysosomal storage disorders  category

Pathogenic 1
Pathogenic/Likely pathogenic 2

Variant name Variant type GRCH38 location Germline classification Molecular consequence dbSNP_ID Submitter
NM_000543.5(SMPD1):c.1252C>T (p.Arg418Ter) Single nucleotide variant Chr11:6393376 Pathogenic Nonsense|non-coding transcript variant|intron variant rs755160837 .Diagnostics Division, CENTRE FOR DNA FINGERPRINTING AND DIAGNOSTICS
.Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
.Neuberg Centre For Genomic Medicine, NCGM
NM_000543.5(SMPD1):c.1276G>A (p.Gly426Ser) Single nucleotide variant Chr11:6393629 Pathogenic/Likely pathogenic Missense variant|non-coding transcript variant rs1554935136 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_000543.5(SMPD1):c.688C>T (p.Arg230Cys) Single nucleotide variant Chr11:6391753 Pathogenic/Likely pathogenic Missense variant|5 prime UTR variant|non-coding transcript variant rs989639224 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics

Indian Institutes & Variant Submission Overview
Chromosome-wise Variant Distribution