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.
References:
- 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.
Glutaric acid (Vitamin B2)
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