123743-99-7

b-Hydroxyisovaleric Acid-d8 is the isotope labelled analog of b-Hydroxyisovaleric Acid (H943605); a metabolite of the essential amino acid Leucine. It is synthesized in the human body and studies suggest that b-Hydroxyisovaleric Acid may have an effect on increasing muscle weight and strength. The presence of elevated b-Hydroxyisovaleric Acid is a sensitive marker of biotin deficiency and may indicate the presence of a genetic disorder such as biotinidase deficiency or holocarboxylase synthetase deficiency.

ChEBI: 3-hydroxyisovaleric acid is a 3-hydroxy monocarboxylic acid that is isovaleric acid substituted at position 3 by a hydroxy group. Used as indicator of biotin deficiency. It has a role as a human metabolite. It is functionally related to a butyric acid and an isovaleric acid. It is a conjugate acid of a 3-hydroxyisovalerate.