GenTIGSA Gene Database on Rare Genetic Disorders

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

L-2-hydroxyglutaric aciduria

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

Pathogenic 1
Pathogenic/Likely pathogenic 1
Uncertain significance 1

Variant name Variant type GRCH38 location Germline classification Molecular consequence dbSNP_ID Submitter
NM_024884.3(L2HGDH):c.1A>G (p.Met1Val) Single nucleotide variant Chr14:50312150 Pathogenic Missense variant|initiator_codon_variant|5 prime UTR variant|intron variant rs1477101414 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_024884.3(L2HGDH):c.908T>A (p.Val303Asp) Single nucleotide variant Chr14:50267909 Uncertain significance Missense variant rs2139977737 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_024884.3(L2HGDH):c.256+1G>A Single nucleotide variant Chr14:50302901 Pathogenic/Likely pathogenic Splice donor variant rs150299874 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics

Indian Institutes & Variant Submission Overview
Chromosome-wise Variant Distribution