4317 3-Hydroxyisovaleric acid 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). 625-08-1 69362 C5H10O3 White powder. 65 - 67°C 3-Hydroxyisovaleric acid is a normal human metabolite excreted in the urine. Elevated levels of this compound are found in several disorders due to impairment of renal reclamation of biotin. Levels may also be increased from prolonged consumption of raw egg-whites. No indication of carcinogenicity to humans (not listed by IARC). This is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation. Chronically high levels of 3-hydroxyisovaleric acid are associated with at least 4 inborn errors of metabolism including: 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency, 3-Methylglutaconic Aciduria Type I, Biotinidase deficiency and Isovaleric Aciduria. 2014-08-29T06:09:43Z 2026-04-14T20:14:43Z 37084 DB15344 true CC(C)(O)CC(O)=O C5H10O3 InChI=1S/C5H10O3/c1-5(2,8)3-4(6)7/h8H,3H2,1-2H3,(H,6,7) AXFYFNCPONWUHW-UHFFFAOYSA-N 118.1311 118.062994186 Endogenous Solid HMDB00754 62571 Rudney, Harry. Biosynthesis of b-hydroxy-b-methylglutaric acid. Journal of Biological Chemistry (1957), 227 363-77. CHEM003223 DTXSID20211535 3-hydroxy-3-methylbutanoic acid