A multimer (24-mer) of this enzyme forms the core of the multienzyme complex, and binds tightly both EC 1.2.4.2, oxoglutarate dehydrogenase (succinyl-transferring) and EC 1.8.1.4, dihydrolipoyl dehydrogenase. The lipoyl group of this enzyme is reductively succinylated by EC 1.2.4.2, and the only observed direction catalysed by EC 2.3.1.61 is that where this succinyl group is passed to coenzyme A.
A multimer (24-mer) of this enzyme forms the core of the multienzyme complex, and binds tightly both EC 1.2.4.2, oxoglutarate dehydrogenase (succinyl-transferring) and EC 1.8.1.4, dihydrolipoyl dehydrogenase. The lipoyl group of this enzyme is reductively succinylated by EC 1.2.4.2, and the only observed direction catalysed by EC 2.3.1.61 is that where this succinyl group is passed to coenzyme A.
enzyme deficient mice show reduced mRNA and protein levels and decreased brain mitochondrial alpha-ketoglutarate dehydrogenase activity (by about 40%), increased vulnerability to mitochondrial toxins: MPTP treatment enhances the severity of lipid peroxidation in the substantial nigra, reduced striatal dopamine (59% depletion in wild-type, 73% in enzyme deficient mutants), dopaminergic neurons (25% reduction in wild-type, 42% in mutants) and tyrosine hydroxylase-positive neurons, striatal lesions induced by malonate (mimicking Huntington's disease, 2fold larger lesions, smaller striatum) or 3-nitropropionic acid (5fold larger lesions than in wild-type) are significantly larger in enzyme deficient mice than in the wild-type, and the 3-nitropropionic acid-induced mitochondrial enzyme inhibition (25% lower citrate synthase activity than in wild-type), protein and DNA oxidation is enhanced in the cortex of enzyme deficient mice compared to wild-type
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
EXPRESSION
ORGANISM
UNIPROT
LITERATURE
enzyme protein levels significantly decrease in wild type mice after 4 weeks of angiotensin-II or 6 weeks of transversal aortic constriction. Enzyme levels significantly decrease in microRNA-146a wild-type mice on angiotensin-II administration
Heggermont, W.A.; Papageorgiou, A.P.; Quaegebeur, A.; Deckx, S.; Carai, P.; Verhesen, W.; Eelen, G.; Schoors, S.; van Leeuwen, R.; Alekseev, S.; Elzenaar, I.; Vinckier, S.; Pokreisz, P.; Walravens, A.S.; Gijsbers, R.; Van Den Haute, C.; Nickel, A.; Schroen, B.; van Bilsen, M.; Janssens, S.; Maack, C.; Pinto, Y.; Carmeliet, P.; Heymans, S.
Inhibition of microRNA-146a and overexpression of its target dihydrolipoyl succinyltransferase protect against pressure overload-induced cardiac hypertrophy and dysfunction