|Other Names||Succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial, ATP-specific succinyl-CoA synthetase subunit beta, Renal carcinoma antigen NY-REN-39, Succinyl-CoA synthetase beta-A chain, SCS-betaA, SUCLA2|
|Format||The synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml deionized water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Catalyzes the ATP-dependent ligation of succinate and CoA to form succinyl-CoA.|
|Tissue Location||Widely expressed. Not expressed in liver and lung.|
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Provided below are standard protocols that you may find useful for product applications.
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.
Ostergaard, E., et al. Eur. J. Pediatr. 169(2):201-205(2010)Elpeleg, O., et al. Am. J. Hum. Genet. 76(6):1081-1086(2005)Rush, J., et al. Nat. Biotechnol. 23(1):94-101(2005)
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