SUCLA2

Entrez Summary: Succinyl-CoA synthetase (SCS) is a mitochondrial matrix enzyme that acts as a heterodimer, being 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 succinate to succinyl-CoA. 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].
UniProt Summary: ATP-specific succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of ATP and thus represents the only step of substrate-level phosphorylation in the TCA (PubMed:15877282). The beta subunit provides nucleotide specificity of the enzyme and binds the substrate succinate, while the binding sites for coenzyme A and phosphate are found in the alpha subunit (By similarity). {ECO:0000255|HAMAP-Rule:MF_03220, ECO:0000269|PubMed:15877282}.

Pfam Domains GO Terms

Compound Hit Most Correlated Genes in Chemogenomics Tissues where Essential in the Avana Dataset (DepMap 20Q1)

Global Fraction of Cell Lines Where Essential: 2/739

Tissue	Fraction Of Cell Lines Where Essential
1290807.0	0/1
909776.0	0/1
bile duct	0/28
blood	0/28
bone	0/26
breast	0/33
central nervous system	0/56
cervix	0/4
colorectal	0/17
esophagus	0/13
fibroblast	0/1
gastric	0/16
kidney	0/21
liver	0/20
lung	0/75
lymphocyte	0/16
ovary	0/26
pancreas	0/24
peripheral nervous system	0/16
plasma cell	0/15
prostate	0/1
skin	0/24
soft tissue	0/9
thyroid	0/2
upper aerodigestive	0/22
urinary tract	0/29
uterus	0/5

Expression Distribution

ATP-grasp 2
Ligase CoA

succinyl-CoA pathway
uroporphyrinogen III metabolic process
uroporphyrinogen III biosynthetic process
succinate-CoA ligase complex
succinate-CoA ligase (ADP-forming) activity
succinyl-CoA metabolic process
succinate metabolic process
porphyrin-containing compound biosynthetic process
tetrapyrrole biosynthetic process
tricarboxylic acid cycle
porphyrin-containing compound metabolic process
tetrapyrrole metabolic process
aerobic respiration
acyl-CoA metabolic process
dicarboxylic acid metabolic process
thioester metabolic process
purine nucleoside bisphosphate metabolic process
nucleoside bisphosphate metabolic process
ribonucleoside bisphosphate metabolic process
cellular respiration
cofactor biosynthetic process
magnesium ion binding
energy derivation by oxidation of organic compounds
coenzyme metabolic process
carboxylic acid biosynthetic process
organic acid biosynthetic process
purine ribonucleotide metabolic process
ribonucleotide metabolic process
purine nucleotide metabolic process
ribose phosphate metabolic process
sulfur compound metabolic process
purine-containing compound metabolic process
mitochondrial matrix
generation of precursor metabolites and energy
cofactor metabolic process
nucleotide metabolic process
nucleoside phosphate metabolic process
drug metabolic process
nucleobase-containing small molecule metabolic process
small molecule biosynthetic process