LP14515-8
Isobutyrylglycine
Active
Description
Isobutyrylglycine is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism. In certain cases the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation. Acyl glycines are produced through the action of glycine N-acyltransferase (EC 2.3.1.13) which is an enzyme that catalyzes the chemical reaction: acyl-CoA + glycine < -- > CoA + N-acylglycine Isobutyrylglycine is identified in large amount in urine of patients with isobutyryl-CoA dehydrogenase deficiency. Isobutyryl-CoA dehydrogenase deficiency is a disorder caused by the deficiency of isobutyryl-CoA dehydrogenase that is involved in the catabolism of the branched-chain amino acid valine PMID: 15505379. 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, Isobutyrylglycine
Basic Part Properties
- Part Display Name
- Isobutyrylglycine
- Part Type
- Component (Describes the core component or analyte measured)
- Created On
- 2000-05-04
- Construct for LOINC Short Name
- Isobutyrylgly
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Language Variants Get Info
| Tag | Language | Translation |
|---|---|---|
| zh-CN | Chinese (China) | 异丁酰甘氨酸 |
| fr-CA | French (Canada) | Isobutyrylglycine |
| et-EE | Estonian (Estonia) | Isobutürüülglütsiin |
| es-ES | Spanish (Spain) | Isobutirilglicina |
| it-IT | Italian (Italy) | Isobutirilglicina |
| tr-TR | Turkish (Turkey) | İzobütirilglisin |
| ru-RU | Russian (Russian Federation) | Изобутирилглицин |
| nl-NL | Dutch (Netherlands) | isobutyrylglycine |
| fr-BE | French (Belgium) | Isobutyrylglycine |
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