Rabbit anti-Human SUCLA2 Polyclonal Antibody

Uniprot Id

Q9P2R7

Target Species

Human

Target Name

SUCLA2

Target Full Name

Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial

Target Function

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. 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.

Target Involvement

Mitochondrial DNA depletion syndrome 5 (MTDPS5)

Target Subcellular Location

Mitochondrion.

Target Protein Families

Succinate/malate CoA ligase beta subunit family, ATP-specific subunit beta subfamily

Target Tissue Specificity

Widely expressed. Not expressed in liver and lung.

Target Research Area

Cardiovascular

Target Synonyms

A BETA; A SCS; ATP specific succinyl CoA synthetase subunit beta; ATP specific succinyl CoA synthetase, beta subunit; ATP-specific succinyl-CoA synthetase subunit beta; Mitochondrial succinyl CoA ligase [ADP forming] subunit beta; MTDPS5; Renal carcinoma antigen NY-REN-39; Renal carcinoma antigen NYREN39; SCS betaA; SCS-betaA; SUCB1_HUMAN; Succinate CoA ligase (ADP forming); Succinate CoA ligase [ADP forming] subunit beta, mitochondrial, succinyl CoA ligase [ADP forming] subunit beta, mitochondrial; Succinate CoA ligase ADP forming beta subunit; Succinate CoA ligase beta subunit; Succinyl CoA ligase [ADP-forming] subunit beta, mitochondrial; Succinyl CoA synthetase beta A chain; Succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial; Succinyl-CoA synthetase beta-A chain; SUCLA2

Target Background

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.