SUCLA2 Antibody (A-9): sc-374107

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SUCLA2 (succinate-CoA ligase, ADP-forming, beta subunit), also known as A-BETA, SCS-betaA or renal carcinoma antigen NY-REN-39, is a 463 amino acid mitochondrial matrix enzyme that belongs to the succinate/malate CoA ligase beta subunit family. Widely expressed, SUCLA2 dimerizes with the SCS alpha subunit to form SCS-A, an essential component of the tricarboxylic acid cycle. Defects in SUCLA2 may be involved in a group of autosomal recessive disorders known as Mitochondrial DNA depletion syndromes (MDSs) that are characterized by a decrease in mitochondrial DNA copy numbers in affected tissues. Progressive external ophthalmoplegia (PEO), ataxia-neuropathy and mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) may also be associated with mutations in SUCLA2. Two isoforms of SUCLA2 exists due to alternative splicing events.