Aliases for SUCLG1 Gene
- Succinate-CoA Ligase Alpha Subunit 2 3 5
- Succinyl-CoA Synthetase Subunit Alpha 3 4
- EC 184.108.40.206 4 56
- SCS-Alpha 3 4
- Succinate--CoA Ligase [ADP/GDP-Forming] Subunit Alpha, Mitochondrial 3
- Succinyl-CoA Ligase [ADP/GDP-Forming] Subunit Alpha, Mitochondrial 3
- Succinyl-CoA Ligase [GDP-Forming] Subunit Alpha, Mitochondrial 3
- Succinate-CoA Ligase, GDP-Forming, Alpha Subunit 2
External Ids for SUCLG1 Gene
Previous GeneCards Identifiers for SUCLG1 Gene
This gene encodes the alpha subunit of the heterodimeric enzyme succinate coenzyme A ligase. This enzyme is targeted to the mitochondria and catalyzes the conversion of succinyl CoA and ADP or GDP to succinate and ATP or GTP. Mutations in this gene are the cause of the metabolic disorder fatal infantile lactic acidosis and mitochondrial DNA depletion. [provided by RefSeq, Feb 2010]
GeneCards Summary for SUCLG1 Gene
SUCLG1 (Succinate-CoA Ligase Alpha Subunit) is a Protein Coding gene. Diseases associated with SUCLG1 include Mitochondrial Dna Depletion Syndrome 9 and Succinate-Coa Ligase Deficiency. Among its related pathways are Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. and Pyruvate metabolism and Citric Acid (TCA) cycle. Gene Ontology (GO) annotations related to this gene include GTP binding. An important paralog of this gene is ACLY.
UniProtKB/Swiss-Prot for SUCLG1 Gene
Succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of either ATP or GTP and thus represents the only step of substrate-level phosphorylation in the TCA. The alpha subunit of the enzyme binds the substrates coenzyme A and phosphate, while succinate binding and specificity for either ATP or GTP is provided by different beta subunits.