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3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate

3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate Struktur
31023-24-2
CAS-Nr.
31023-24-2
Englisch Name:
3-(3-methylbutanoyloxy)-4-trimethylammonio-butanoate
Synonyma:
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
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
Optische Aktivit?t
[α]/D -23±2°, c = 1 in H2O
BRN 
5946880

Sicherheit

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( 26)Lieferanten
Firmenname Telefon E-Mail Land Produktkatalog Edge Rate
TargetMol Chemicals Inc.
+1-781-999-5354 +1-00000000000
marketing@targetmol.com United States 32161 58
Dayang Chem (Hangzhou) Co.,Ltd.
571-88938639 +8617705817739
info@dycnchem.com China 52849 58
GIHI CHEMICALS CO.,LIMITED
+8618058761490
info@gihichemicals.com China 49978 58
Aladdin Scientific
+1-+1(833)-552-7181
sales@aladdinsci.com United States 57505 58
Sigma-Aldrich 021-61415566 800-8193336
orderCN@merckgroup.com China 51456 80
AN PharmaTech Co Ltd 86(21)68097365
sales@anpharma.net China 4891 55
Shanghai EFE Biological Technology Co., Ltd. 021-65675885 18964387627
info@efebio.com China 9806 58
Shanghai Rechem science Co., Ltd. 21-31433387 15618786686
sales@rechemscience.com China 2988 58
Guangzhou Younan Technology Co., Ltd 020-82000279 18988968278
sales@ubiochem.com China 4297 58
ShangHai Anpel Co, Ltd. 18501792038; 18501792038
shanpel@anpel.com.cn China 9611 58

  • 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
  • Isovaleryl-L-carnitine >=97.0% (TLC)
  • (R)-3-((3-methylbutanoyl)oxy)-4-(trimethylammonio)butanoate(WXG00321)
  • isoverylcarnitine.
  • 31023-24-2
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