ID:SUCA_HUMAN DESCRIPTION: RecName: Full=Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial; EC=6.2.1.4; EC=6.2.1.5; AltName: Full=Succinyl-CoA synthetase subunit alpha; Short=SCS-alpha; Flags: Precursor; FUNCTION: 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). CATALYTIC ACTIVITY: GTP + succinate + CoA = GDP + phosphate + succinyl-CoA. CATALYTIC ACTIVITY: ATP + succinate + CoA = ADP + phosphate + succinyl-CoA. PATHWAY: Carbohydrate metabolism; tricarboxylic acid cycle. SUBUNIT: Heterodimer of an alpha and a beta subunit. SUBCELLULAR LOCATION: Mitochondrion. DISEASE: Defects in SUCLG1 are the cause of mitochondrial DNA depletion syndrome type 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. SIMILARITY: Belongs to the succinate/malate CoA ligase alpha subunit family. SEQUENCE CAUTION: Sequence=AAD17940.2; Type=Erroneous initiation; Sequence=AAH00504.1; Type=Erroneous initiation; Sequence=CAA92426.1; Type=Erroneous initiation;
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on P53597
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
GeneReviews article(s) related to gene SUCLG1: suclg1-mtddepl (SUCLG1-Related Mitochondrial DNA Depletion Syndrome, Encephalomyopathic Form with Methylmalonic Aciduria)
Gene Model Information
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Methods, Credits, and Use Restrictions
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US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
