alpha-ketoglutarate dehydrogenase

(redirected from Oxoglutarate dehydrogenase)
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α-ke·to·glu·tar·ate de·hy·dro·gen·ase

an enzyme that catalyzes the oxidative decarboxylation of 2-ketoglutaric acid to succinyldihydrolipoate; the succinyl group is later transferred to CoA and the reduced lipoate is oxidized by NAD+; a complex that is a part of the tricarboxylic acid cycle.
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References in periodicals archive ?
Our patient with hyperglycemia and dysmorphic features had a deletion of 7.23 Mb comprising the region 7p13-p12.1, with involvement of 39 Online Mendelian Inheritance in Man genes, including glucokinase associated with maturity-onset diabetes of the young, CCM2 associated with type 2 cerebral cavernous malformations, insulin-like growth factors binding protein-3 associated with decreased postnatal growth, and oxoglutarate dehydrogenase associated with alpha-ketoglutarate dehydrogenase deficiency (short stature, hypotonia, cognitive impairment, and movement abnormalities).