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3-Hydroxyisovalerylcarnitine

CAT: 0804-HY-N11200Size: 1 mgDry Ice: NoHazardous: No
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CAT#:0804-HY-N11200Size:1 mg
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24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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Description
3-Hydroxyisovalerylcarnitine is a carnitine derivative. 3-Hydroxyisovaleryl carnitine in plasma may serve as a novel biomarker of biotin deficiency in humans, with its concentration increasing with biotin deficiency. Biotin deficiency reduces the activity of biotin-dependent 3-methylcrotonyl-CoA carboxylase, hinders the conversion of 3-methylcrotonyl-CoA into 3-methylglutaconyl-CoA, and impairs the leucine catabolism pathway; resulting in plasma 3-Hydroxyisovalerylcarnitine concentrations rise[1].
CAS Number
99159-87-2
UNSPSC
12352211
Hazard Statement
H302, H315, H319, H335
Target
Endogenous Metabolite
Type
Natural Products
Related Pathways
Metabolic Enzyme/Protease
Applications
Metabolism-protein/nucleotide metabolism
Field of Research
Metabolic Disease
Assay Protocol
https://www.medchemexpress.com/3-hydroxyisovalerylcarnitine.html
Purity
99.40
Solubility
H2O : 1 mg/mL (ultrasonic; warming) |DMSO : 50 mg/mL
Smiles
CC(O)(CC(O[C@@H](C[N+](C)(C)C)CC([O-])=O)=O)C
Molecular Formula
C12H23NO5
Molecular Weight
261.31
Precautions
H302, H315, H319, H335
References & Citations
[1]Horvath TD, et al. Quantitative measurement of plasma 3-hydroxyisovaleryl carnitine by LC-MS/MS as a novel biomarker of biotin status in humans. Anal Chem. 2010 May 15;82 (10) :4140-4.|[2]Maeda Y, et al., Determination of 3-hydroxyisovalerylcarnitine and other acylcarnitine levels using liquid chromatography-tandem mass spectrometry in serum and urine of a patient with multiple carboxylase deficiency. J Chromatogr B Analyt Technol Biomed Life Sci. 2008 Jul 15;870 (2) :154-9.
Shipping Conditions
Blue Ice
Storage Conditions
-20°C (Powder, protect from light)
Scientific Category
Natural Products
Clinical Information
No Development Reported

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