2-ketoadipic acid

2-ketoadipic acid
An intermediate in l-tryptophan and l-lysine catabolism; k. accumulates in certain inherited disorders, probably due to a deficiency of one of the proteins in the α-ketoadipate dehydrogenase complex; 2-oxoadipic acid; 2-oxohexadioic acid.
- 2-ketoadipic acid dehydrogenase complex the multienzyme complex that reacts k. with coenzyme A and NAD+ to produce glutaryl-CoA, CO2, and NADH + H+ in l-lysine and l-tryptophan catabolism; a deficiency of one of the proteins in this complex results in 2-ketoadipic acidemia.

Medical dictionary. 2011.

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  • α-ketoadipic acid — α ke·to·adip·ic ac·id (ke″to ə dipґik) a dicarboxylic acid formed by transamination of α aminoadipic acid in the degradation of lysine and hydroxylysine. Written also 2 ketoadipic acid …   Medical dictionary

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  • α-ketoadipate — α ke·to·adip·ate (ke″to ə dipґāt) the anionic form of α ketoadipic acid …   Medical dictionary

  • α-ketoadipate dehydrogenase — α ke·to·adip·ate de·hy·dro·gen·ase (ke″to ə dipґāt de hiґdro jən ās) a mitochondrial enzyme activity that catalyzes the decarboxylation and esterification of α ketoadipic acid, forming glutaryl CoA as a step in …   Medical dictionary

  • α-ketoadipicaciduria — α ke·to·adip·ic·ac·id·uria (ke″to ə dip″ik as″ĭ duґre ə) excretion of α ketoadipic acid in the urine …   Medical dictionary

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