数据之源，洞见之始

Metabolite Card

Note

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. beta-Hydroxyisovaleric acid is under investigation in clinical trial NCT03018496 (Investigating the Effects of Beta-Hydroxy-Beta-Methylbutyrate on Glucose Handling in Older and Younger Men.). beta-Hydroxyisovaleric acid is a natural product found in Aloe africana, Arabidopsis thaliana, and other organisms with data available. Juven is an orally bioavailable nutritional supplement. Juven contains the amino acids glutamine and arginine in addition to beta-hydroxy-beta-methylbutyrate (HMB). This agent may promote muscle protein synthesis and increase muscle mass. (NCI04) 3-Hydroxyisovaleric acid is a normal human metabolite excreted in the urine. Elevated levels of this compound are found in several inherited disorders such as Dihydrolipoamide dehydrogenase Deficiency, 3-Methylcrotonyl-CoA carboxylase 1 deficiency, 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency (3-hydroxy-3-methylglutaryl -CoA lyase Deficiency, Biotinidase deficiency multiple carboxylase deficiency late-onset , Late onset multiple carboxylase deficiency, HolMcarboxylase synthetase deficiency, 3-Methylcrotonyl-CoA carboxylase 2 deficiency. 3-Hydroxyisovaleric acid is also elevated in smokers, in subjects undergoing long-term anticonvulsant therapy with carbamazepine and/or phenytoin. These levels are elevated due to impairment of renal reclamation of biotin. Levels may also be increased from prolonged consumption of raw egg-whites (OMIM: 210210, 253270, 600529, 253260, 246450, 210200, 238331). (A3355, A3356, A3357, A3358).