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Glutaminyl-tRNA amidotransferase subunit A, mitochondrial (QRSL1)
- Target
- QRSL1
- Molecular classification
- Enzyme, Mitochondrial aminoacyl-tRNA amidotransferase complex (specifically, GatA subunit of GatCAB complex)[1][3]
01
Overview
Glutaminyl-tRNA amidotransferase subunit A, mitochondrial (QRSL1), is the GatA subunit of the GatCAB complex, located in the mitochondria of human cells[1][4][5][6]. This enzyme enables *indirect* charging of mitochondrial tRNA(Gln) with glutamine through transamidation: initially, mt-tRNA Gln is charged with glutamic acid, which is then converted to glutaminyl-tRNA(Gln) in a reaction requiring glutamine and ATP. This step is critical for proper mitochondrial protein synthesis and the assembly of oxidative phosphorylation complexes. Genetic variants in QRSL1 disrupt mitochondrial translation, leading to mitochondrial diseases characterized by cardiac and metabolic dysfunction, notably combined oxidative phosphorylation deficiency 40[3]. It is a protein-coding gene with no known directly interacting therapeutic drugs and is not currently established as a common drug target, although its dysfunction has clear pathophysiological significance.
- Other names
- QRSL1GatAGlutaminyl-tRNA synthase (glutamine-hydrolyzing)-like 1Glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrialGlutaminyl-tRNA amidotransferase subunit COXPD40Glutaminyl-tRNA synthase-like protein 1Glu-AdT subunit AGATADKFZP564C1278FLJ10989FLJ12189FLJ13447
02
Mechanism of action
Not established or detailed in current literature. No drugs directly targeting QRSL1 reported.
03
Biological functions
Mitochondrial protein translationGlutaminyl-tRNAGln biosynthesis via transamidation[1][5]Correct aminoacylation of Gln-tRNA(Gln) in mitochondria[1][3][5]Indirect charging of mitochondrial tRNA(Gln) (mt-tRNA Gln) with glutamine—converts misacylated Glu-tRNA(Gln) into Gln-tRNA(Gln)[3][1]
04
Disease associations
Mitochondrial diseaseCombined oxidative phosphorylation deficiency 40Metabolic cardiomyopathyDisorders of mitochondrial translation or protein synthesis[1][3][5]
05
Safety considerations
Genetic defects in QRSL1 lead to severe metabolic dysfunctions, such as combined oxidative phosphorylation deficiency and mitochondrial cardiomyopathy, resulting in often fatal outcomes if not managed[3].Tissue-specific variation in symptoms and severity; some mutations lead to complete loss of function or variable residual activity, complicating diagnostics and potential therapeutic intervention[3].
06
Biomarkers
Pathogenic QRSL1 variants may serve as biomarkers for certain mitochondrial translation disorders and combined oxidative phosphorylation deficiency 40[3].
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