GenTIGSA Gene Database on Rare Genetic Disorders

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

Propionic acidemia

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

Conflicting classifications of pathogenicity 1
Pathogenic 1
Pathogenic/Likely pathogenic 2

Variant name Variant type GRCH38 location Germline classification Molecular consequence dbSNP_ID Submitter
NM_000282.4(PCCA):c.1682T>G (p.Leu561Arg) Single nucleotide variant Chr13:100368510 Conflicting classifications of pathogenicity Missense variant|non-coding transcript variant rs2152811823 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_000282.4(PCCA):c.1426C>T (p.Arg476Ter) Single nucleotide variant Chr13:100309905 Pathogenic/Likely pathogenic Nonsense|non-coding transcript variant rs768703749 .Institute of Medical Genetics and Genomics, Sir Ganga Ram Hospital
.Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_000532.5(PCCB):c.494G>A (p.Arg165Gln) Single nucleotide variant Chr3:136262016 Pathogenic Missense variant rs1304714042 .Lifecell International Pvt. Ltd
NM_000532.5(PCCB):c.1228C>T (p.Arg410Trp) Single nucleotide variant Chr3:136327184 Pathogenic/Likely pathogenic Missense variant rs121964959 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India

Indian Institutes & Variant Submission Overview
Chromosome-wise Variant Distribution