LP15301-2
3-Hydroxyisovalerate
Active
Description
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 PMID: 16895887, PMID: 9523856, PMID: 15447901, PMID: 9176832, [OMIM: 210210], [OMIM: 253270], [OMIM: 600529], [OMIM: 253260], [OMIM: 246450], [OMIM: 210200], [OMIM: 238331] Copyright Copyright ©2005-2009 Genome Alberta (Reference to original publication: Wishart DS, Knox C, Guo AC, et al. HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2009 37(Database issue):D603-610.) Source: Human Metabolome Database, Human Metabolome Database
Basic Part Properties
- Part Display Name
- 3-Hydroxyisovalerate
- Part Type
- Component (Describes the core component or analyte measured)
- Created On
- 2000-05-04
- Construct for LOINC Short Name
- 3OH-Isovalerate
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Language Variants Get Info
| Tag | Language | Translation |
|---|---|---|
| zh-CN | Chinese (China) | 3-羟基异戊酸 Synonyms: 3-HIA; |
| fr-CA | French (Canada) | 3-hydroxyisovalérate |
| et-EE | Estonian (Estonia) | 3-hüdroksüisovaleraat |
| es-ES | Spanish (Spain) | 3-Hidroxiisovalerato |
| it-IT | Italian (Italy) | 3-Idrossiisovalerato |
| tr-TR | Turkish (Turkey) | 3-Hidroksiizovalerat |
| ru-RU | Russian (Russian Federation) | 3-Гидроксиизовалерат |
| nl-NL | Dutch (Netherlands) | 3-hydroxyisovaleraat |
| fr-BE | French (Belgium) | 3-hydroxyisovalérate |
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