Glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial (GATB)

Glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial (GATB), is a mitochondrial protein-coding enzyme that serves as the B subunit of the *glutamyl-tRNA(Gln) amidotransferase* (Glu-AdT, also known as the GatCAB complex) [3][1]. This multimeric complex is essential for the formation of correctly charged glutaminyl-tRNA(Gln) in mitochondria via a transamidation reaction that modifies misacylated glutamyl-tRNA(Gln), thereby ensuring fidelity during mitochondrial protein translation[1][3]. GATB, together with GatA and GatC, catalyzes the conversion of Glu-tRNA(Gln) to Gln-tRNA(Gln) in the presence of glutamine and ATP[3][1][4]. GATB has structural homology with Pet112-like proteins, which are conserved from bacteria to humans and are essential for mitochondrial function[1]. Pathogenic mutations in GATB disrupt mitochondrial translation, causing combined oxidative phosphorylation deficiency and other severe mitochondrial translation disorders[3][4]. GATB is not a direct pharmacological drug target, and no interacting drugs or mechanisms of action are currently reported.

Other names

Glutamyl-tRNA amidotransferase subunit BPET112PET112LHSPC199Glu-AdT subunit BCytochrome c oxidase assembly factor PET112 homologCOXPD41Glutamyl-tRNA(Gln) amidotransferase, subunit BPET112 homologcytochrome oxidase assembly factor PET112 homolog