Isovalerylglycine is produced from leucine catabolism. It is further metabolized via isovaleryl-CoA dehydrogenase. This enzyme requires vitamin B2 as a cofactor.
- Gregersen N. Riboflavin-responsive defects of beta-oxidation. J Inher Metab Dis. 1985;8 Suppl 1:65-69.
- Finocchiaro G, Ito M, Tanaka K. Purification and properties of short chain acyl-CoA, medium chain acyl-CoA, and isovaleryl-CoA dehydrogenases from human liver. J Biol Chem. 1987;262(17):7982-7989.
- Bei F, Sun JH, Yu YG, et al. Two novel isovaleryl-CoA dehydrogenase gene mutations in a Chinese infant. Gene. 2013;524(2):396-400.
- Merritt JL, 2nd, Norris M, Kanungo S. Fatty acid oxidation disorders. Ann Transl Med. 2018;6(24):473-473.
- Sahai I, Garganta CL, Bailey J, et al. Newborn Screening for Glutaric Aciduria-II: The New England Experience. JIMD reports. 2014;13:1-14.
- Manoli I, Venditti CP. Disorders of branched chain amino acid metabolism. Transl Sci Rare Dis. 2016;1(2):91-110.
- Chinen Y, Nakamura S, Tamashiro K, et al. Isovaleric acidemia: Therapeutic response to supplementation with glycine, l-carnitine, or both in combination and a 10-year follow-up case study. Mol Genet Metab Rep. 2017;11:2-5.
- Shigematsu Y, Sudo M, Momoi T, Inoue Y, Suzuki Y, Kameyama J. Changing plasma and urinary organic acid levels in a patient with isovaleric acidemia during an attack. Ped Res. 1982;16(9):771-775.
- Capo-chichi CD, Guéant J-L, Lefebvre E, et al. Riboflavin and riboflavin-derived cofactors in adolescent girls with anorexia nervosa. Am J Clin Nutr. 1999;69(4):672-678.
Acyl-CoA dehydrogenase enzymes are not only involved in branched-chain amino acid metabolism, but also beta-oxidation of fatty acids.
Enzymatic dysfunction and elevations in isovalerylglycine are seen when there is a functional nutrient cofactor need and in certain inborn errors of metabolism. However, elevations of isovalerylglycine are also seen in problematic mitochondrial fatty acid beta-oxidation.
Carnitine, glycine, vitamin B2, and antioxidants have been used therapeutically to treat abnormal levels of isovalerylglycine.
There is an association between elevated isovalerylglycine and anorexia nervosa. The mechanism is believed to be due to poor thyroid conversion of vitamin B2 into active FAD, which normalized in some patients after a refeeding program.
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