3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate
- CAS No.
- 31023-24-2
- Chemical Name:
- 3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate
- 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
- CBNumber:
- CB11236041
- Molecular Formula:
- C12H23NO4
- Molecular Weight:
- 245.32
- MDL Number:
- MOL File:
- 31023-24-2.mol
- MSDS File:
- SDS
| storage temp. | 2-8°C |
|---|---|
| solubility | Soluble in DMSO |
| optical activity | [α]/D -23±2°, c = 1 in H2O |
| BRN | 5946880 |
| FDA UNII | CJS2BGX9TF |
3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate price More Price(8)
| Manufacturer | Product number | Product description | CAS number | Packaging | Price | Updated | Buy |
|---|---|---|---|---|---|---|---|
| Sigma-Aldrich | 51371 | Isovaleryl-L-carnitine ≥94.0% (HPLC) | 31023-24-2 | 10MG | $90.4 | 2023-06-20 | Buy |
| Sigma-Aldrich | 51371 | Isovaleryl-L-carnitine ≥94.0% (HPLC) | 31023-24-2 | 50MG | $357 | 2023-06-20 | Buy |
| Sigma-Aldrich | 91403 | Isovaleryl-L-carnitine analytical standard | 31023-24-2 | 10MG | $106 | 2022-05-15 | Buy |
| Sigma-Aldrich | 91403 | Isovaleryl-L-carnitine analytical standard | 31023-24-2 | 50MG | $371 | 2022-05-15 | Buy |
| American Custom Chemicals Corporation | CHM0094535 | 3-(3-METHYLBUTANOYLOXY)-4-TRIMETHYLAMMONIO-BUTANOATE 95.00% | 31023-24-2 | 10MG | $709.46 | 2021-12-16 | Buy |
3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate Chemical Properties,Uses,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
3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate Suppliers
| Supplier | Tel | Country | ProdList | Advantage | |
|---|---|---|---|---|---|
| TargetMol Chemicals Inc. | +1-781-999-5354 +1-00000000000 | marketing@targetmol.com | United States | 19892 | 58 |
| Dayang Chem (Hangzhou) Co.,Ltd. | 571-88938639 +8617705817739 | info@dycnchem.com | CHINA | 52867 | 58 |
| GIHI CHEMICALS CO.,LIMITED | +8618058761490 | info@gihichemicals.com | China | 49999 | 58 |
| Meike meditech (Foshan) Co.,Ltd | +86-0757-22239986 +86-18029359586 | sales@mkmeditech.com | China | 1134 | 58 |
| Aladdin Scientific | +1-833-552-7181 | sales@aladdinsci.com | United States | 57511 | 58 |
| Sigma-Aldrich | 021-61415566 800-8193336 | orderCN@merckgroup.com | China | 51471 | 80 |
| AN PharmaTech Co Ltd | 86(21)68097365 | sales@anpharma.net | China | 4901 | 55 |
| Shanghai Rechem science Co., Ltd. | 21-31433387 15618786686 | sales@rechemscience.com | China | 2985 | 58 |
| ShangHai Anpel Co, Ltd. | 18501792038; 18501792038 | shanpel@anpel.com.cn | China | 9614 | 58 |
| Energy Chemical | 021-58432009 400-005-6266 | marketing@energy-chemical.com | China | 44941 | 58 |