| Identification | Back Directory | [Name]
3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate | [CAS]
31023-24-2 | [Synonyms]
Isovalerylcarnitine
Isovaleroyl Carnitine
O-isovalerylcarnitine
ISOVALERYL-L-CARNITINE
3-methylbutyrylcarnitine
Isovaleryl-L-carnitine >=97.0% (TLC)
3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate
(3R)-3-(3-Methylbutanoyloxy)-4-(trimethylazaniumyl)butanoate
(R)-3-((3-methylbutanoyl)oxy)-4-(trimethylammonio)butanoate(WXG00321)
(R)-3-Carboxy-N,N,N-trimethyl-2-(3-methyl-1-oxobutoxy)-1-propanaminium inner salt | [Molecular Formula]
C12H23NO4 | [MOL File]
31023-24-2.mol | [Molecular Weight]
245.32 |
| Chemical Properties | Back Directory | [storage temp. ]
2-8°C | [solubility ]
Soluble in DMSO | [form ]
Solid | [color ]
White to off-white | [Appearance]
white solid | [Optical Rotation]
[α]/D -23±2°, c = 1 in H2O | [BRN ]
5946880 |
| Hazard Information | Back Directory | [Description]
(C5) Isovalerylcarnitine is an analogue of endogenous acylcarnitine, formed in the body as a result of leucine metabolism, and has an isovaleric acid residue as an acyl substituent. The profile of acylcarnitines in blood and tissues is used as a marker of various conditions associated with mitochondrial (and/or peroxisomal) dysfunction.
The product is used primarily as a control for MS/MS.
Isovalerylcarnitine — the main biochemical marker for isovaleric acidemia, which occurs as a result of deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase. Isovalerylcarnitine has been found to be associated with celiac disease and very long chain acyl-CoA dehydrogenase deficiency (VLCAD). Also, the level of isovalerylcarnitine is determined when studying neuralgia, for example, with trigeminal neuralgia. | [Uses]
Isovaleryl-L-carnitine may be used as an analytical reference standard for the quantification of the analyte in biological samples using chromatography-based techniques. | [Definition]
ChEBI: O-isovalerylcarnitine is a C5-acylcarnitine having isovaleryl as the acyl substituent. It has a role as a human metabolite. It is functionally related to an isovaleric acid. | [General Description]
Isovaleryl-L-carnitine belongs to the acylcarnitines class of compounds. Acyl-L-carnitines are mostly produced from L-carnitine, which plays an important role in fatty acid metabolic pathways. The functions and roles of acyl-L-carnitines in various tissues like brain, heart, and muscle continue to attract much interest, since a number of diseases caused by defects of mitochondrial transport are characterized by specific metabolic dysfunctions and depend on the physiological role of the affected carrier in intermediary metabolism. | [Biochem/physiol Actions]
Isovalerylcarnitine is the phenotypic abnormality in iso-valeric acidemia resulting from an accumulation of isovaleric acid, which is toxic to the central nervous system. Isovaleric acidemia is an autosomal recessive inborn error of leucine metabolism caused by a deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase resulting in the accumulation of derivatives of isovaleryl-CoA. It was the first organic acidemia recognized in humans and can cause significant morbidity and mortality. Early diagnosis and treatment with a protein restricted diet and supplementation with carnitine and glycine are effective in promoting normal development in severely affected individuals. An alternative pathway through glycine-N-acylase allows detoxification by producing isovaleryl-glycine, which is excreted. Thus, isovalerylcarnitine and isovalerylglycine are the hallmarks of this disorder in plasma and urine, respectively, and are elevated regardless of a patient′s metabolic condition. | [IC 50]
Human Endogenous Metabolite | [storage]
Store at -20°C |
|
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Sigma-Aldrich
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Energy Chemical
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http://www.energy-chemical.com |
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