51371

Isovaleryl-L-carnitine

≥94.0% (HPLC)

• Synonym(s): (2R)-3-Carboxy-N,N,N-trimethyl-2-(3-methyl-1-oxobutoxy)-1-propanaminium inner salt, L-Carnitine isovaleryl ester, iC5-Carnitine
• Empirical Formula (Hill Notation): C₁₂H₂₃NO₄
• CAS Number: 31023-24-2
• Molecular Weight: 245.32
• Beilstein: 5946880
• PubChem Substance ID: 329757684
• NACRES: NA.26

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 Information

• Storage Class Code: 13 - Non Combustible Solids
• WGK: WGK 3
• Flash Point(F): Not applicable
• Flash Point(C): Not applicable