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| 3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate Basic information |
| Product Name: | 3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate | | Synonyms: | 3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate;Isovalerylcarnitine;3-methylbutyrylcarnitine;Isovaleroyl Carnitine;(3R)-3-(3-Methylbutanoyloxy)-4-(trimethylazaniumyl)butanoate;(R)-3-Carboxy-N,N,N-trimethyl-2-(3-methyl-1-oxobutoxy)-1-propanaminium inner salt;ISOVALERYL-L-CARNITINE;O-isovalerylcarnitine | | CAS: | 31023-24-2 | | MF: | C12H23NO4 | | MW: | 245.32 | | EINECS: | | Product Categories: | | Mol File: | 31023-24-2.mol |
| 3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate Chemical Properties |
| storage temp. | 2-8°C | | optical activity | [α]/D -23±2°, c = 1 in H2O | | BRN | 5946880 |
| 3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate Usage And Synthesis |
| 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. | | 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. |
| 3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate Preparation Products And Raw materials |
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