3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate
- Product Name
- 3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate
- CAS No.
- 31023-24-2
- Chemical Name
- 3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate
- Synonyms
- isoverylcarnitine.;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)
- CBNumber
- CB11236041
- Molecular Formula
- C12H23NO4
- Formula Weight
- 245.32
- MOL File
- 31023-24-2.mol
3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate Property
- storage temp.
- 2-8°C
- solubility
- Soluble in DMSO
- form
- Solid
- color
- White to off-white
- optical activity
- [α]/D -23±2°, c = 1 in H2O
- BRN
- 5946880
N-Bromosuccinimide Price
- Product number
- 51371
- Product name
- Isovaleryl-L-carnitine
- Purity
- ≥94.0% (HPLC)
- Packaging
- 10MG
- Price
- $90.4
- Updated
- 2023/06/20
- Product number
- 51371
- Product name
- Isovaleryl-L-carnitine
- Purity
- ≥94.0% (HPLC)
- Packaging
- 50MG
- Price
- $357
- Updated
- 2023/06/20
- Product number
- 91403
- Product name
- Isovaleryl-L-carnitine
- Purity
- analytical standard
- Packaging
- 10MG
- Price
- $106
- Updated
- 2022/05/15
- Product number
- 91403
- Product name
- Isovaleryl-L-carnitine
- Purity
- analytical standard
- Packaging
- 50MG
- Price
- $371
- Updated
- 2022/05/15
- Product number
- CHM0094535
- Product name
- 3-(3-METHYLBUTANOYLOXY)-4-TRIMETHYLAMMONIO-BUTANOATE
- Purity
- 95.00%
- Packaging
- 10MG
- Price
- $709.46
- Updated
- 2021/12/16
3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate Chemical Properties,Usage,Production
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.
3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate Preparation Products And Raw materials
Raw materials
Preparation Products
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