Beta Aminoisobutyric Acid

Optimal Result: 0 - 3 umol/L.

Beta-aminoisobutyric acid (also known as 3-aminoisobutyric acid) is a non-protein amino acid formed by the catabolism of valine and the nucleotide thymine. It is further catabolized to methylmalonic acid semialdehyde and propionyl-CoA. Levels are controlled by a vitamin B6-dependent reaction in the liver and kidneys. β-aminoisobutyric acid can also be produced by skeletal muscle during physical activity.


- Van Kuilenburg AB, Stroomer AE, Van Lenthe H, Abeling NG, Van Gennip AH. New insights in dihydropyrimidine dehydrogenase deficiency: a pivotal role for betaaminoisobutyric acid? Biochem J. 2004;379(Pt 1):119-124.

- Tanianskii DA, Jarzebska N, Birkenfeld AL, O’Sullivan JF, Rodionov RN. Beta-Aminoisobutyric Acid as a Novel Regulator of Carbohydrate and Lipid Metabolism. Nutrients. 2019;11(3).

- Roberts LD, Bostrom P, O’Sullivan JF, et al. betaAminoisobutyric acid induces browning of white fat and hepatic beta-oxidation and is inversely correlated with cardiometabolic risk factors. Cell Metab. 2014;19(1):96-108.

What does it mean if your Beta Aminoisobutyric Acid result is too low?

Low levels of β-AIB may be seen with decreased precursors, such as valine. Dihydropyrimidine dehydrogenase deficiency is a rare inborn error of metabolism that results in lower levels of urinary β-AIB.

What does it mean if your Beta Aminoisobutyric Acid result is too high?

Elevated levels may be associated with increased intake of the precursor amino acid valine. Levels are higher with exercise.

A functional need for vitamin B6 can also contribute to elevations. Clinically, transient high levels have been observed under a variety of pathological conditions including lead poisoning, starvation, total body irradiation, and malignancy.

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