Optimal Result: 0 - 1.7 mmol/mol creatinine.

α-Ketoadipic Acid (AKAA; 2-Oxoadipic acid, 2-Ketoadipic acid) is an organic acid formed from α-aminoadipic acid (which originates with lysine) and also from α-aminomuconic acid (derived from tryptophan).

AKAA metabolizes to form glutaryl-CoA via oxidative decarboxylation. The cofactors needed in this step are Coenzyme A, NAD, thiamine pyrophosphate (vitamin B1), lipoic acid, and vitamin B2.


- Wilson R, Wilson C, Gates S, Higgins J. α-Ketoadipic aciduria: A description of a new metabolic error in lysine-tryptophan degradation. Ped Res. 1975;9(6):522-526.

- Bender DA, Njagi EN, Danielian PS. Tryptophan metabolism in vitamin B6-deficient mice. Br J Nutr. 1990;63(1):27-36.

- Solmonson A, DeBerardinis RJ. Lipoic acid metabolism and mitochondrial redox regulation. J Biol Chem. 2018;293(20):7522- 7530.

- Dougherty FE. Metabolic testing in mitochondrial disease. Paper presented at: Seminars in neurology2001.

What does it mean if your a-Ketoadipate result is too high?

Elevations in urinary AKAA may reflect enzymatic dysfunction due to nutritional cofactor needs.

Mitochondrial oxidative phosphorylation disorders are also associated with higher levels of AKAA.

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