GenTIGSA Gene Database on Rare Genetic Disorders

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

Pyruvate carboxylase deficiency

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

Likely pathogenic 2
Pathogenic 1

Variant name Variant type GRCH38 location Germline classification Molecular consequence dbSNP_ID Submitter
NM_022172.2(PC):c.[2095G>A];[2095G>T] CompoundHeterozygote Chr: Likely pathogenic .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_001040716.2(PC):c.2668G>T (p.Val890Phe) Single nucleotide variant Chr11:66850270 Pathogenic Missense variant rs1555014957 .Department of Medical Genetics, Sanjay Gandhi Post Graduate Institute of Medical Sciences
NM_001040716.2(PC):c.1368+1G>A Single nucleotide variant Chr11:66863773 Likely pathogenic Splice donor variant rs755640269 .GenePathDx, GenePath diagnostics

Indian Institutes & Variant Submission Overview
Chromosome-wise Variant Distribution