GenTIGSA Gene Database on Rare Genetic Disorders

Variants in ClinVar from 582 of 1004 Rare Genetic Disorders of GenTIGS

GM1 gangliosidosis type 2

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

Conflicting classifications of pathogenicity 1
Pathogenic 4
Pathogenic/Likely pathogenic 2
Uncertain significance 1

Variant name Variant type GRCH38 location Germline classification Molecular consequence dbSNP_ID Submitter
NM_000404.4(GLB1):c.253C>A (p.Pro85Thr) Single nucleotide variant Chr3:33068963 Uncertain significance Missense variant|intron variant rs1575472038 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_000404.4(GLB1):c.495_497del (p.Leu166del) Deletion Chr3:33065518 - 33065520 Conflicting classifications of pathogenicity Inframe_deletion rs754077128 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_000404.4(GLB1):c.1325G>A (p.Arg442Gln) Single nucleotide variant Chr3:33018470 Pathogenic/Likely pathogenic Missense variant rs564428355 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
.Neuberg Centre For Genomic Medicine, NCGM
NM_000404.4(GLB1):c.276G>A (p.Trp92Ter) Single nucleotide variant Chr3:33068940 Pathogenic Nonsense|intron variant rs748830051 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_000404.4(GLB1):c.1769G>A (p.Arg590His) Single nucleotide variant Chr3:32997310 Pathogenic/Likely pathogenic Missense variant|intron variant rs398123351 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_000404.4(GLB1):c.202C>T (p.Arg68Trp) Single nucleotide variant Chr3:33072587 Pathogenic Missense variant rs72555370 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_000404.4(GLB1):c.1369C>T (p.Arg457Ter) Single nucleotide variant Chr3:33016819 Pathogenic Nonsense rs72555359 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
.Neuberg Centre For Genomic Medicine, NCGM
NM_000404.4(GLB1):c.75+2dup Duplication Chr3:33097008 - 33097009 Pathogenic 5 prime UTR variant|splice donor variant rs587776525 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
.Neuberg Centre For Genomic Medicine, NCGM

Indian Institutes & Variant Submission Overview
Chromosome-wise Variant Distribution