Anti-SUCLA2 Antibody Picoband® Fluoro647 Conjugated

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, 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.

Mediator of RNA polymerase II transcription subunit 14; Activator-recruited cofactor 150 kDa component; ARC150; Cofactor required for Sp1 transcriptional activation subunit 2; CRSP complex subunit 2; Mediator complex subunit 14; RGR1 homolog; hRGR1; Thyroid hormone receptor-associated protein complex 170 kDa component; Trap170; Transcriptional coactivator CRSP150; Vitamin D3 receptor-interacting protein complex 150 kDa component; DRIP150; MED14; ARC150, CRSP2, CXorf4, DRIP150, EXLM1, RGR1, TRAP170

Component of the Mediator complex, a coactivator involved in the regulated transcription of nearly all RNA polymerase II-dependent genes. Mediator functions as a bridge to convey information from gene-specific regulatory proteins to the basal RNA polymerase II transcription machinery. Mediator is recruited to promoters by interactions with regulatory proteins and serves as a scaffold for the assembly of a functional preinitiation complex with RNA polymerase II and the general transcription factors.

1. Carrozzo, R., Dionisi-Vici, C., Steuerwald, U., Lucioli, S., Deodato, F., Di Giandomenico, S., Bertini, E., Franke, B., Kluijtmans, L. A. J., Meschini, M. C., Rizzo, C., Piemonte, F., Rodenburg, R., Santer, R., Santorelli, F. M., van Rooij, A., Vermunt-de Koning, D., Morava, E., Wevers, R. A. SUCLA2 mutations are associated with mild methylmalonic aciduria, Leigh-like encephalomyopathy, dystonia, and deafness. Brain 130: 862-874, 2007. 2. Elpeleg, O., Miller, C., Hershkovitz, E., Bitner-Glindzicz, M., Bondi-Rubinstein, G., Rahman, S., Pagnamenta, A., Eshhar, S., Saada, A. Deficiency of the ADP-forming succinyl-CoA synthase activity is associated with encephalomyopathy and mitochondrial DNA depletion. Am. J. Hum. Genet. 76: 1081-1086, 2005. 3. Gross, M. B. Personal Communication. Baltimore, Md. 5/29/2015.