Optimal Result: 0 - 0.51 mmol/mol creatinine.

Glutarate (Glutaric Acid) is formed from the essential amino acids lysine and tryptophan through the intermediaries of alpha ketoadipic acid and glutaryl-CoA. Glutaryl-CoA is further metabolized to glutaconyl- and crotonyl-CoA by an enzyme called glutaryl-CoA dehydrogenase. This enzyme requires riboflavin (vitamin B2) as a cofactor.


- Beresford MW, Pourfarzam M, Turnbull DM, Davidson JE. So doctor, what exactly is wrong with my muscles? Glutaric aciduria type II presenting in a teenager. Neuromusc Dis 2006;16(4):269- 273.

- Behin A, Acquaviva-Bourdain C, Souvannanorath S, et al. Multiple acyl-CoA dehydrogenase deficiency (MADD) as a cause of lateonset treatable metabolic disease. Rev Neurolog. 2016;172(3):231- 241.

- Chalmers RA, Bain MD, Zschocke J. Riboflavin-responsive glutaryl CoA dehydrogenase deficiency. Mol Genet Metab. 2006;88(1):29- 37.

- Chokchaiwong S, Kuo Y-T, Lin S-H, et al. Coenzyme Q10 serves to couple mitochondrial oxidative phosphorylation and fatty acid β-oxidation, and attenuates NLRP3 inflammasome activation. Free Rad Res. 2018;52(11-12):1445-1455.

What does it mean if your Glutarate result is too high?

Elevations of urinary Glutarate (glutaric acid) may reflect enzymatic insufficiency requiring vitamin B2 or mitochondrial electron transport dysfunction. Deficiencies of the enzyme glutaryl-CoA dehydrogenase, and multiple acyl-CoA dehydrogenase deficiency (MADD), are well-studied inborn errors of metabolism which result in significant glutaric aciduria. However, milder forms of this rare mitochondrial disorder exist and can result in adult-onset presentations. Late-onset forms can present as atypical beta-oxidation disorders with exercise intolerance, muscle weakness, and CNS dysfunction. In these cases, riboflavin, carnitine, and CoQ10 have been used therapeutically.

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