QRSL1

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Description

The QRSL1 (glutaminyl-tRNA amidotransferase subunit QRSL1) is a protein-coding gene located on chromosome 6.

QRSL1 plays a crucial role in the mitochondria by ensuring accurate charging of Gln-tRNA(Gln). It achieves this through a transamidation process, converting incorrectly charged Glu-tRNA(Gln) into the correct Gln-tRNA(Gln). This process requires the presence of glutamine and ATP and involves an activated gamma-phospho-Glu-tRNA(Gln) intermediate.

QRSL1 is also known as COXPD40, GatA.

Associated Diseases

• Combined oxidative phosphorylation deficiency 40