QRSL1

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).

Glutaminyl-TRNA Synthase (Glutamine-Hydrolyzing)-Like 1

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 transamidationManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process mitochondrial translationManual Assertion Based On ExperimentIMP:UniProtKB
Biological Process regulation of protein stabilityIEA:Ensembl

Mitochondrion

Combined oxidative phosphorylation deficiency 40 (COXPD40):
An autosomal recessive mitochondrial disorder characterized by prenatal or infantile onset, fetal hydrops, severe hypertrophic cardiomyopathy, poor growth, sensorineural hearing loss, hepatic dysfunction, lactic acidosis, and decreased activities of mitochondrial respiratory complexes I, III, IV, and V. The disorder is lethal, with death occurring in infancy.