91298

3-Hydroxyisovaleryl-L-carnitine

analytical standard

• Synonym(s): (2R)-3-Carboxy-2-(3-hydroxy-3-methyl-1-oxobutoxy)-N,N,N-trimethyl-1-propanaminium inner salt
• Empirical Formula (Hill Notation): C₁₂H₂₃NO₅
• CAS Number: 99159-87-2
• Molecular Weight: 261.31
• Beilstein: 10549761

Properties

• grade: analytical standard
• Quality Level: 100
• Assay: ≥90.0% (HPLC)
• optical activity: [α]/D -17.0±2.0°, c = 0.1 in H₂O
• shelf life: limited shelf life, expiry date on the label
• impurities: ≤15% water (calc. on elemental analysis)
• application(s): clinical testing
• format: neat
• storage temp.: 2-8°C
• SMILES string: C[N+](C)(C)C[C@H](OC(CC(C)(O)C)=O)CC([O-])=O
• InChI: 1S/C12H23NO5/c1-9(2)6-11(16)18-12(17,7-10(14)15)8-13(3,4)5/h9,17H,6-8H2,1-5H3
• InChI key: DHRNMNHPSWBJEN-UHFFFAOYSA-N

Description

Biochem/physiol Actions

3-Hydroxyisovaleryl-L-carnitine is an endogenous metabolite that can result from impairment in the leucine catabolic pathway. 3-Hydroxyisovaleryl-L-carnitine was observed in plasma and urine of patient with isolated 3-methylcrotonyl-CoA carboxylase deficiency and is used as biomarker of biotin deficiency in humans and other metabolic disorders.

Safety Information

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