3oxoadipate

3-oxoadipate, or 3-oxoadipic acid, is a six-carbon dicarboxylic keto acid that serves as an intermediate in the mitochondrial degradation of certain amino acids. Its structure features a keto group on the third carbon of the adipate backbone, with carboxyl groups at both termini. In biological systems, 3-oxoadipate is typically encountered in its CoA-activated form, 3-oxoadipyl-CoA, during the breakdown of lysine, hydroxylysine, and tryptophan.

In cellular metabolism, 3-oxoadipyl-CoA is converted by the oxoadipate dehydrogenase complex in a reaction that connects amino-acid catabolism to central energy pathways. The reaction yields acetyl-CoA and succinyl-CoA, which feed into the citric acid cycle and other energy-producing processes. This makes 3-oxoadipate a pivotal link between amino-acid degradation and general metabolism.

Occurrence and detection are primarily studied in the context of physiology and pathology. Under normal conditions, trace amounts of 3-oxoadipate and related metabolites are detectable in body fluids and may be measured by techniques such as gas chromatography–mass spectrometry or high-performance liquid chromatography.

Clinical relevance is most notable in rare metabolic disorders. 3-oxoadipic aciduria is an inherited condition arising from defects in the oxoadipate dehydrogenase complex, leading to elevated levels of 3-oxoadipate and related compounds in urine. Symptoms can vary and may include developmental delay, hypotonia, and metabolic disturbances. Diagnosis relies on urinary organic acid profiling, and management often involves dietary adjustments to limit lysine and tryptophan intake, along with supportive therapies such as carnitine supplementation in some cases.

See also: lysine degradation, tryptophan degradation, oxoadipate dehydrogenase.