GATB

GATB (Glutamyl-TRNA Amidotransferase Subunit B) is a protein coding gene. Among its related pathways are tRNA Aminoacylation and Metabolism. Gene Ontology annotations related to this gene include ligase activity and translation factor activity, RNA binding.

Full Name

Glutamyl-TRNA Amidotransferase Subunit B

Function

Allows the formation of correctly charged Gln-tRNA(Gln) through the transamidation of misacylated Glu-tRNA(Gln) in the mitochondria. The reaction takes place in the presence of glutamine and ATP through an activated gamma-phospho-Glu-tRNA(Gln).

Biological Process

Glutaminyl-tRNAGln biosynthesis via transamidation Source: UniProtKB
Mitochondrial translation Source: UniProtKB

Cellular Location

Mitochondrion

Involvement in disease

Combined oxidative phosphorylation deficiency 41 (COXPD41):
An autosomal recessive mitochondrial disorder characterized by prenatal onset, fetal hydrops, intrauterine growth retardation, hypertrophic cardiomyopathy, respiratory insufficiency, lactic acidosis, and decreased activities of mitochondrial respiratory complexes I, III, IV, and V. The disorder is lethal, with death occurring in the perinatal period.