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MGP003695

UniProt Annotations

Entry Information
Gene Namesuccinate-CoA ligase, alpha subunit
Protein EntrySUCA_HUMAN
UniProt IDP53597
SpeciesHuman
Comments
Comment typeDescription
Catalytic ActivityATP + succinate + CoA = ADP + phosphate + succinyl-CoA.
Catalytic ActivityGTP + succinate + CoA = GDP + phosphate + succinyl-CoA.
DiseaseMitochondrial DNA depletion syndrome 9 (MTDPS9) [MIM:245400]: A severe disorder due to mitochondrial dysfunction. It is characterized by infantile onset of hypotonia, lactic acidosis, severe psychomotor retardation, progressive neurologic deterioration, and excretion of methylmalonic acid. {ECO:0000269|PubMed:17668387, ECO:0000269|PubMed:19526370, ECO:0000269|PubMed:20453710, ECO:0000269|PubMed:20693550}. Note=The disease is caused by mutations affecting the gene represented in this entry.
FunctionCatalyzes 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). {ECO:0000250}.
PathwayCarbohydrate metabolism; tricarboxylic acid cycle.
Sequence CautionSequence=AAD17940.2; Type=Erroneous initiation; Evidence={ECO:0000305}; Sequence=AAH00504.1; Type=Erroneous initiation; Evidence={ECO:0000305}; Sequence=CAA92426.1; Type=Erroneous initiation; Evidence={ECO:0000305};
SimilarityBelongs to the succinate/malate CoA ligase alpha subunit family. {ECO:0000305}.
Subcellular LocationMitochondrion.
SubunitHeterodimer of an alpha and a beta subunit.
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