3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate
|
|
|
- CAS-Nr.
- 31023-24-2
- Englisch Name:
- 3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate
- Synonyma:
- Isovalerylcarnitine;ISOVALERYL-L-CARNITINE;isoverylcarnitine.;O-isovalerylcarnitine;Isovaleroyl Carnitine;3-methylbutyrylcarnitine;Isovaleryl-L-carnitine;Isovaleryl-L-carnitine >=97.0% (TLC);3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate;(R)-3-((3-Methylbutanoyl)oxy)-4-(trimethylammonio)butanoate
- CBNumber:
- CB11236041
- Summenformel:
- C12H23NO4
- Molgewicht:
- 245.32
- MOL-Datei:
- 31023-24-2.mol
|
3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate Eigenschaften
- storage temp.
- 2-8°C
- L?slichkeit
- Soluble in DMSO
- Aggregatzustand
- Solid
- Farbe
- White to off-white
- Aussehen
- white solid
- Optische Rotation
- [α]/D -23±2°, c = 1 in H2O
- BRN
- 5946880
- InChI
- 1S/C12H23NO4/c1-9(2)6-12(16)17-10(7-11(14)15)8-13(3,4)5/h9-10H,6-8H2,1-5H3/t10-/m1/s1
- InChIKey
- IGQBPDJNUXPEMT-SNVBAGLBSA-N
- SMILES
- C[N+](C)(C)C[C@H](OC(CC(C)C)=O)CC([O-])=O
Sicherheit
| WGK Germany |
WGK 3 |
|
|
| Speicherklasse |
11 - Combustible Solids |
|
|
3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate Chemische Eigenschaften,Einsatz,Produktion Methoden
Verwenden
Isovaleryl-L-carnitine may be used as an analytical reference standard for the quantification of the analyte in biological samples using chromatography-based techniques.
Allgemeine Beschreibung
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 Upstream-Materialien And Downstream Produkte
Upstream-Materialien
Downstream Produkte
3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate Anbieter Lieferant Produzent Hersteller Vertrieb H?ndler.
Global( 46)Lieferanten
- 3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate
- 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
- O-isovalerylcarnitine
- Isovaleryl-L-carnitine >=97.0% (TLC)
- (R)-3-((3-methylbutanoyl)oxy)-4-(trimethylammonio)butanoate(WXG00321)
- isoverylcarnitine.
- Isovaleryl-L-carnitine
- (R)-3-((3-Methylbutanoyl)oxy)-4-(trimethylammonio)butanoate
- Isovalerylcarnitine
- ISOVALERYL-L-CARNITINE
- 31023-24-2
