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.
Low levels of glutaric acid, which can indicate a deficiency in riboflavin (vitamin B2), have various implications. Riboflavin is an essential nutrient that plays a crucial role in energy metabolism and cellular function. A deficiency in riboflavin can lead to various health issues, including fatigue, skin disorders, and problems with the nervous system. Glutaric acid is used as an indicator in laboratory tests to identify riboflavin deficiency. It's important to address low glutaric acid levels by increasing the intake of foods rich in vitamin B2 or by taking riboflavin supplements as recommended by a healthcare professional. Riboflavin deficiency is treatable and can be managed effectively with dietary adjustments and supplementation.
Elevations of urinary 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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