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.
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.
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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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1-Methylhistidine, 3-Methylhistidine, a-Amino-N-butyric Acid, a-Aminoadipic Acid, Alanine, Arginine, Asparagine, Aspartic Acid, b-Alanine, b-Aminoisobutyric Acid, Citrulline, Cyst(e)ine, Cystathionine, Ethanolamine, g-Aminobutyric Acid, Glutamic Acid, Glutamine, Glycine, Histidine, Isoleucine, Leucine, Lysine, Methionine, Ornithine, Phenylalanine, Phosphoethanolamine, Phosphoserine, Proline, Sarcosine, Serine, Taurine, Threonine, Tryptophan, Tyrosine, Urea, Valine