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.
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.
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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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3,4-Dihydroxyphenylpropionate, 3-Methyl-4-OH-phenylglycol, 5-Hydroxyindoleacetate, 8-Hydroxy-2-deoxyguanosine, a-Hydroxybutyrate, a-Hydroxyisobutyrate, a-Keto-b-Methylvalerate, a-Ketoadipate, a-Ketoglutarate, a-Ketoisocaproate, a-Ketoisovalerate, a-Ketophenylacetate, Adipate, b-Hydroxybutyrate, b-Hydroxyisovalerate, b-Hydroxypropionate, Benzoate, Cis-Aconitate, Citramalate, Citrate, Creatinine, D-Arabinitol, Formiminoglutamate, Glutarate, Glycerate, Glycolate, Hippurate, Homovanillate, Hydroxymethylglutarate, Indoleacetate, Isocitrate, Isovalerylglycine, Kynurenate, Kynurenate/Quinolinate, Lactate, m-Hydroxyphenylacetate, Malate, Methylmalonate, Orotate, Oxalate, p-Hydroxyphenylacetate, Phenylacetate, Pyroglutamate, Pyruvate, Quinolinate, Suberate, Succinate, Tartarate, Vanilmandelate, Xanthurenate
