SUCLA2

SUCLA2
Identifiers
Aliases	SUCLA2, A-BETA, MTDPS5, SCS-betaA, succinate-CoA ligase ADP-forming beta subunit, A-SCS, succinate-CoA ligase ADP-forming subunit beta, LINC00444
External IDs	OMIM: 603921; MGI: 1306775; HomoloGene: 2856; GeneCards: SUCLA2; OMA:SUCLA2 - orthologs
Gene location (Human)
Chr.	Chromosome 13 (human)
End	48,037,968 bp
Gene location (Mouse)
Chr.	Chromosome 14 (mouse)
End	73,833,582 bp
RNA expression pattern
	Top expressed in
	jejunal mucosa; ; Pons; ; lateral nuclear group of thalamus; ; body of tongue; ; vastus lateralis muscle; ; biceps brachii; ; right ventricle; ; Skeletal muscle tissue of rectus abdominis; ; thoracic diaphragm; ; myocardium of left ventricle;
	Top expressed in
	atrioventricular valve; ; intercostal muscle; ; seminiferous tubule; ; spermatid; ; facial motor nucleus; ; cardiac muscle tissue of left ventricle; ; vastus lateralis muscle; ; masseter muscle; ; triceps brachii muscle; ; extraocular muscle;
	More reference expression data
	n/a
Gene ontology
Molecular function	nucleotide binding; ligase activity; protein binding; catalytic activity; ATP binding; metal ion binding; succinate-CoA ligase (ADP-forming) activity; magnesium ion binding;
Cellular component	myelin sheath; mitochondrial matrix; extracellular exosome; mitochondrion; succinate-CoA ligase complex;
Biological process	succinate metabolic process; metabolism; tricarboxylic acid cycle; succinyl-CoA pathway; succinyl-CoA metabolic process;
	Sources:Amigo / QuickGO
Orthologs
	8803
	20916
	ENSG00000136143
	ENSMUSG00000022110
	Q9P2R7; Q5T9Q8
	Q9Z2I9
	NM_003850
	NM_011506; NM_001361638
	NP_003841
	NP_035636; NP_001348567
	Wikidata
View/Edit Human	View/Edit Mouse

Succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial (SUCLA2), also known as ADP-forming succinyl-CoA synthetase (SCS-A), is an enzyme that in humans is encoded by the SUCLA2 gene on chromosome 13.

Succinyl-CoA synthetase (SCS) is a mitochondrial matrix enzyme that acts as a heterodimer, composed of an invariant alpha subunit and a substrate-specific beta subunit. The protein encoded by this gene is an ATP-specific SCS beta subunit that dimerizes with the SCS alpha subunit to form SCS-A, an essential component of the tricarboxylic acid cycle. SCS-A hydrolyzes ATP to convert succinyl-CoA to succinate. Defects in this gene are a cause of myopathic mitochondrial DNA depletion syndrome. A pseudogene of this gene has been found on chromosome 6. [provided by RefSeq, Jul 2008]