TARS2

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Description

The TARS2 (threonyl-tRNA synthetase 2, mitochondrial) is a protein-coding gene located on chromosome 1.

TARS2 catalyzes the addition of threonine to tRNA(Thr) in a two-step process. Firstly, threonine is activated by ATP to create Thr-AMP. Then, Thr-AMP is transferred to the acceptor end of tRNA(Thr). TARS2 also has an editing domain that corrects any mischarged tRNA(Thr).

TARS2 is also known as COXPD21, TARSL1, thrRS.

Associated Diseases

• Combined oxidative phosphorylation deficiency 21