Isovalerylglycine

Optimal result: 0 – 3.7 mmol/mol creatinine

Genova Diagnostics

By Healthmatters Editorial Team

Peer-reviewed sources

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.

Isovalerylglycine is produced from leucine catabolism. It is further metabolized via isovaleryl-CoA dehydrogenase. This enzyme requires vitamin B2 as a cofactor.

References:

- 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.

Organize and understand all your lab results

Start organizing — $15/mo →

For Health Professionals Healthmatters Pro Multiple clients’ results in one dashboard. See Pro plans →

Isovalerylglycine

Every lab result you’ve ever had, charted and explained.

Healthmatters is a tracker for your lab results. Upload a report from any lab, any country — every marker charted and explained.

Start with my report →

Clinical Sources & References

All our content is backed by peer-reviewed studies, academic research, and trusted medical sources. We’re committed to accuracy and transparency — see our editorial policy for details.

Article Review Log

Written by Healthmatters Editorial Team

Organize and understand all your lab results

Start organizing — $15/mo →

For Health Professionals Healthmatters Pro Multiple clients’ results in one dashboard. See Pro plans →

Healthmatters Lifetime Plan

Pay $250. Track your health forever

One payment. No renewals. Yours for life.

10,000+ biomarkers explained · trends over time

See other plans →

Secure checkout via Stripe · GDPR & HIPAA

We accept results from any lab

LabCorp Quest Diagnostics Genova Diagnostics DUTCH Test Doctor's Data Vibrant America ZRT Laboratory Cyrex Labs + any other lab

What members are saying

★★★★★ 10,000+ happy members

★★★★★

"Watching my numbers move over the years — instead of forgetting last time's results — completely changed how I manage my health."

Daniel

Member since 2022

★★★★★

"Living across several countries made keeping my lab results organized a real challenge. Healthmatters brought my results from the USA, Spain, and Germany together — 20 years of history, with long-term trends instead of scattered reports."

Ben

Member since 2021 · tracks across countries

★★★★★

"Standard lab ranges are too wide for how I practice — 'normal' isn't optimal. I set my own bands, so a client sees yellow before a marker goes officially out of range. That's the conversation I want to be having."

Dr. Alexandra

Functional medicine · 6 yrs on Pro