SUCLA2 cDNA ORF Clone in Cloning Vector, Human

SUCLA2 encodes the ATP-forming β subunit (A-SUCL-β) of succinyl-CoA ligase, an enzyme of the citric acid cycle. Sucla2 is located in the mitochondrial matrix and catalyzes the reversible synthesis of succinate and adenosine triphosphate (ATP) in the tricarboxylic acid cycle. Mutations in SUCLA2, encoding the ß-subunit of succinyl-CoA synthetase of the Krebs cycle, are one cause of mitochondrial DNA depletion syndrome. Mutations in SUCLA2 lead to a mitochondrial disorder manifesting as encephalomyopathy with dystonia, deafness, and lesions in the basal ganglia. Patients have been reported to have severe progressive childhood-onset encephalomyopathy, and methylmalonic aciduria, often leading to death in childhood. Sucla2 expression was found to be correlated with the capacitation of boar spermatozoa. The metabolic vulnerability in SUCLA2-deficient prostate cancer cells is pharmacologically targetable.

succinate-CoA ligase, ADP-forming, beta subunit

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