13766-1
Isovalerylglycine/Creatinine [Mass Ratio] in Urine
Active
Part Descriptions
LP14516-6 Isovalerylglycine
Isovalerylglycine 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 Isovalerylglycine is a byproduct of the catabolism of the aminoacid leucine. Accumulation of isovalerylglycine occurs in Isovaleric Acidemia (IVA). IVA (OMIM/ McKusick 243500) is an autosomal recessive disorder caused by mutations in the isovaleryl-CoA dehydrogenase (EC 1.3.99.10) gene. The deficiency of this enzyme in the metabolism of leucine leads to the accumulation of a series of isovaleryl-CoA metabolites, such as isovalerylglycine (IVG). It is very important to caution for false positive results when screening for isovaleric acidemia by tandem mass spectrometry based on dried blood-spot levels of C5-acylcarnitines, including isovalerylcarnitine and its isomer, pivaloylcarnitine; pivaloylcarnitine is derived from pivalate-generating antibiotics, and has caused many false-positive results. PMID: 17850781.
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, Isovalerylglycine
LP32035-5 Creatinine
Creatinine or creatine anhydride, is a breakdown product of creatine phosphate in muscle. The loss of water molecule from creatine results in the formation of creatinine. It is transferred to the kidneys by blood plasma, whereupon it is eliminated by glomular filtration and partial tubular excretion. Creatinine is usually produced at a fairly constant rate and measuring its serum level is a simple test. A rise in blood creatinine levels is observed only with marked damage to functioning nephrons; therefore this test is not suitable for detecting early kidney disease. Creatine and creatinine are metabolized in the kidneys, muscle, liver and pancreas.
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, Creatinine
Fully-Specified Name
- Component
- Isovalerylglycine/Creatinine
- Property
- MRto
- Time
- Pt
- System
- Urine
- Scale
- Qn
- Method
Additional Names
- Short Name
- Isovalerylgly/Creat Ur
- Display Name
- Isovalerylglycine/Creatinine (U) [Mass ratio]
- Consumer Name Alpha Get Info
- Isovalerylglycine/Creatinine, Urine
Basic Attributes
- Class
- CHEM
- Type
- Laboratory
- First Released
- Version 1.0k
- Last Updated
- Version 2.73
- Change Reason
- This term was Discouraged in a previous release because at the time (2013), this analyte was not being measured in mass units. We are changing the term back to Active status because at least two major laboratories are currently (in 2018) measuring this analyte in mass units.
- Order vs. Observation
- Observation
- Common Test Rank Get Info
- 7222
Member of these Panels
| LOINC | Long Common Name |
|---|---|
| 50334-2 | Acylglycines panel [Mass Ratio] - Urine |
Member of these Groups Get Info
| LOINC Group | Group Name |
|---|---|
| LG34610-2 | Isovalerylglycine/Creatinine| |
Language Variants Get Info
| Tag | Language | Translation |
|---|---|---|
| de-DE | German (Germany) | Isovalerylglycin/Creatinin: |
| es-AR | Spanish (Argentina) | isovalerilglicina/creatinina: |
| es-ES | Spanish (Spain) | Isovalerilglicina/Creatinina: Synonyms: Cuantitativo |
| es-MX | Spanish (Mexico) | Isovalerilglicina / Creatinina: |
| et-EE | Estonian (Estonia) | Isovalerüülglütsiin/kreatiniin: Synonyms: Juhuslik Kvantitatiivne Uriin |
| fr-CA | French (Canada) | Isovalérylglycine/Créatinine: |
| fr-BE | French (Belgium) | Isovalérylglycine/Créatinine: Synonyms: Rapport de masse |
| fr-FR | French (France) | Isovalérylglycine/créatinine: |
| it-IT | Italian (Italy) | Isovalerilglicina/Creatinina: Synonyms: Chimica Punto nel tempo (episodio) Rapporto di Massa |
| ko-KR | Korean (Korea, Republic Of) | 이소발레릴글리신/크레아티닌: |
| nl-NL | Dutch (Netherlands) | isovalerylglycine/creatinine: Synonyms: kreatinine |
| pt-BR | Portuguese (Brazil) | Isovalerilglicina/Creatinina: Synonyms: ; |
| ru-RU | Russian (Russian Federation) | Изовалерилглицин/Креатинин: Synonyms: Количественный Масс отношение Точка во времени; |
| tr-TR | Turkish (Turkey) | İzovalerilglisin/Kreatinin: |
| uk-UA | Ukrainian (Ukraine) | Ізовалерилгліцин/Креатинін: Synonyms: Chemistry; |
| zh-CN | Chinese (China) | 异戊酰甘氨酸/肌酐: Synonyms: CR; |
Example Units
| Unit | Source |
|---|---|
| mcg/mg cr | REGENSTRIEF |
| mg/gm cr | REGENSTRIEF |
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