Drug pipeline
Full profile accessExplore the programs pursuing this target and their development progress.
- Drug candidates
- Developers
- Development stage
Target intelligence / Profile preview
Glutamyl-tRNA(Gln) amidotransferase subunit C, mitochondrial (GATC)
- Target
- GATC
- Molecular classification
- Enzyme, Mitochondrial enzyme complex subunit
01
Overview
Glutamyl-tRNA(Gln) amidotransferase subunit C, mitochondrial (GATC) is a mitochondrial protein that forms part of the heterotrimeric GatCAB complex, which is essential for mitochondrial protein synthesis. This enzyme catalyzes the transamidation of misacylated Glu-tRNA(Gln) to correctly charged Gln-tRNA(Gln), compensating for the absence of a dedicated mitochondrial glutaminyl-tRNA synthetase. This pathway is crucial for the fidelity of protein translation in mitochondria. Pathogenic variants in GATC impair mitochondrial translation and lead to severe diseases such as combined oxidative phosphorylation deficiency and metabolic cardiomyopathy. GATC mutation or dysfunction can result in tissue-specific defects, particularly affecting tissues with high metabolic demand (notably heart and muscle), and can be lethal in humans and model organisms.
- Other names
- 15E1.2COXPD42Glu-AdT subunit CFLJ37000Protein 15E1.2glutamyl-tRNA(Gln) amidotransferase subunit C, mitochondrialglu-AdT subunit Cglutamyl-tRNA(Gln) amidotransferase, subunit C homologGATC
02
Biological functions
Mitochondrial translationAminoacyl-tRNA biosynthesis via transamidationAccurate charging of glutamyl-tRNA(Gln)
03
Disease associations
Mitochondrial diseaseCombined oxidative phosphorylation deficiencyMetabolic cardiomyopathy
04
Safety considerations
Not classically targeted by therapeutics; loss-of-function or pathogenic variants cause severe mitochondrial dysfunction, defective translation, and often fatal metabolic disorders
05
Biomarkers
Deficiency in GATC or GatCAB complex for diagnosis of combined oxidative phosphorylation deficiency 42 or related mitochondrial translation defects
Beyond the preview
Go deeper on Glutamyl-tRNA(Gln) amidotransferase subunit C, mitochondrial (GATC).
Explore the evidence, development activity, and competitive landscape with Gosset’s full data platform.
Clinical trials
Full profile accessFollow the clinical studies evaluating therapies directed at this target.
- Trial design
- Status
- Readouts
Competitive landscape
Full profile accessCompare approaches across drug candidates, modalities, and indications.
- Programs
- Modalities
- Indications
Literature & evidence
Full profile accessInvestigate the research and source evidence behind target biology and development.
- Publications
- Sources
- Analysis
Patents
Full profile accessExplore patent activity around therapies and technologies addressing this target.
- Patents
- Assignees
- Technologies
Research & analysis
Full profile accessConnect target biology, drug development, and emerging evidence in your research.
- Biology
- Development news
- Analysis
Bring the full picture into focus.
See how Gosset can support your research on Glutamyl-tRNA(Gln) amidotransferase subunit C, mitochondrial (GATC).