Glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial (or Glu-AdT subunit A) is an enzyme that in humans is encoded by the QRSL1 gene. Also called GatA, this protein combines with the proteins GATB and GATC to form the Glu-tRNA(Gln) amidotransferase complex that is involved in incorporating the amino acid glutamine into proteins as part of protein synthesis in the mitochondria.
Medical significance Pathogenic mutations in the gene QRSL1 are associated with a mitochondrial disease called combined oxidative phosphorylation deficiency-40 (COXPD40). Symptoms typically start at or before birth, and include poor growth, hearing loss, heart problems, and early death.
See also EARS2 – Preceding enzyme in pathway
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