31023-24-2 Usage
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.
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.
Check Digit Verification of cas no
The CAS Registry Mumber 31023-24-2 includes 8 digits separated into 3 groups by hyphens. The first part of the number,starting from the left, has 5 digits, 3,1,0,2 and 3 respectively; the second part has 2 digits, 2 and 4 respectively.
Calculate Digit Verification of CAS Registry Number 31023-24:
(7*3)+(6*1)+(5*0)+(4*2)+(3*3)+(2*2)+(1*4)=52
52 % 10 = 2
So 31023-24-2 is a valid CAS Registry Number.
InChI:InChI=1/C12H23NO4/c1-9(2)6-12(16)17-10(7-11(14)15)8-13(3,4)5/h9-10H,6-8H2,1-5H3
31023-24-2Relevant articles and documents
Differentiation of Isomeric Acylcarnitines Using Tandem Mass Spectrometry
Gaskell, Simon, J.,Guenat, Christian,Millington, David S.,Maltby, David A.,Roe, Charles R.
, p. 2801 - 2805 (2007/10/02)
Tandem mass spectrometry, using a four-sector instrument, has been used to distinguish isomeric acylcarnitines.Collisional activation of MH+ ions (or of acylcarnitine methyl ester cations) induces fragmentations of the acyl moieties, yielding daughter ions with relative abundances characteristic of individual isomers.Direct analyses of crude urine samples from children with metabolic disorders enable unequivocal identification of individual acylcarnitines, thus contributing to the characterization of the disorder.
