Aliases for SUCLG1 Gene
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 Fatal Infantile Lactic Acidosis With Methylmalonic Aciduria. Among its related pathways are Glucose / Energy Metabolism and Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins.. GO annotations related to this gene include poly(A) RNA binding and GTP binding. An important paralog of this gene is ACLY.
UniProtKB/Swiss-Prot for SUCLG1 Gene
Catalyzes the ATP- or GTP-dependent ligation of succinate and CoA to form succinyl-CoA. The nature of the beta subunit determines the nucleotide specificity (By similarity).