91298

3-Hydroxyisovaleryl-L-carnitine

analytical standard

• Synonyme(s) : (2R)-3-Carboxy-2-(3-hydroxy-3-methyl-1-oxobutoxy)-N,N,N-trimethyl-1-propanaminium inner salt
• Formule empirique (notation de Hill): C₁₂H₂₃NO₅
• Numéro CAS: 99159-87-2
• Poids moléculaire : 261.31
• Beilstein: 10549761
• Numéro MDL: MFCD32061910
• Code UNSPSC : 41116107
• Nomenclature NACRES : NA.24

Propriétés

• Qualité: analytical standard
• Niveau de qualité: 100
• Essai: ≥90.0% (HPLC)
• Activité optique: [α]/D -17.0±2.0°, c = 0.1 in H₂O
• Durée de conservation: limited shelf life, expiry date on the label
• Impuretés: ≤15% water (calc. on elemental analysis)
• Application(s): clinical testing
• Format: neat
• Température de stockage: 2-8°C
• Chaîne SMILES: 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
• Clé InChI: DHRNMNHPSWBJEN-UHFFFAOYSA-N

Description

Actions biochimiques/physiologiques

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.

Produits recommandés

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Informations sur la sécurité

• Code de la classe de stockage: 11 - Combustible Solids
• Classe de danger pour l'eau (WGK): WGK 3
• Point d'éclair (°F): Not applicable
• Point d'éclair (°C): Not applicable