Isovalerylcarnitine

• CAT Number: I019796
• CAS Number: 31023-24-2
• Molecular Formula: C₁₂H₂₃NO₄
• Molecular Weight: 245.32
• Purity: ≥95%
• Target: Proteasome
• IUPAC Name: 3-(3-methylbutanoyloxy)-4-(trimethylazaniumyl)butanoate
• InChI: 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
• InChIKey: IGQBPDJNUXPEMT-UHFFFAOYSA-N
• SMILES: CC(C)CC(=O)OC(CC(=O)[O-])C[N+](C)(C)C

Isovalerylcarnitine(CAT: I019796) is an acylcarnitine derivative formed by the conjugation of isovaleric acid with carnitine. It plays a significant role in fatty acid metabolism, facilitating the transport of branched-chain amino acid (BCAA) catabolism byproducts, particularly from leucine metabolism, into mitochondria for β-oxidation. Elevated levels of isovalerylcarnitine are used as a key biomarker in metabolic disease research, especially for diagnosing isovaleric acidemia (IVA), a rare genetic disorder caused by a deficiency in isovaleryl-CoA dehydrogenase. Its analysis aids in understanding metabolic dysfunctions, energy production, and mitochondrial disorders, contributing to the development of diagnostic and therapeutic strategies.