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51371 Sigma-Aldrich

Isovaleryl-L-carnitine

≥94.0% (HPLC)

Synonym: (2R)-3-Carboxy-N,N,N-trimethyl-2-(3-methyl-1-oxobutoxy)-1-propanaminium inner salt, L-Carnitine isovaleryl ester, iC5-Carnitine

  • CAS Number 31023-24-2

  • Empirical Formula (Hill Notation) C12H23NO4

  • Molecular Weight 245.32

  •  Beilstein/REAXYS Number 5946880

  •  PubChem Substance ID 329757684

  •  NACRES NA.26

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Properties

Quality Level   100
assay   ≥94.0% (HPLC)
form   powder or crystals
optical activity   [α]/D -23±2°, c = 1 in H2O
impurities   ≤10% water
color   white to off-white
storage temp.   2-8°C
SMILES string   C[N+](C)(C)C[C@H](OC(CC(C)C)=O)CC([O-])=O
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
InChI key   IGQBPDJNUXPEMT-SNVBAGLBSA-N

Description

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.

Safety & Documentation

Safety Information

RIDADR 
NONH for all modes of transport
WGK Germany 
WGK 3
Flash Point(F) 
Not applicable
Flash Point(C) 
Not applicable

Documents

Certificate of Analysis (COA)

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Protocols & Articles

Articles

Acyl-L-Carnitines

Carnitine was discovered at the beginning of the last century and plays an important role in fatty acid metabolic pathways.1 It acts as a carrier of activated fatty acids across the inner mitochondri...
Keywords: Chromatography, Diseases, High performance liquid chromatography, Liquid chromatography mass spectrometry, Mass spectrometry, Metabolic Pathways, Metabolism, Separation

Peer-Reviewed Papers
15

References

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