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
• UNSPSC Code: 41116107

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.

Recommended products

Find a digital Reference Material for this product available on our online platform ChemisTwin® for NMR. You can use this digital equivalent on ChemisTwin® for your sample identity confirmation and compound quantification (with digital external standard). An NMR spectrum of this substance can be viewed and an online comparison against your sample can be performed with a few mouseclicks. Learn more here and start your free trial.

Safety Information

• Storage Class Code: 11 - Combustible Solids
• WGK: WGK 3
• Flash Point(F): Not applicable
• Flash Point(C): Not applicable