91298

3-Hydroxyisovaleryl-L-carnitine

analytical standard

• 別名: (2R)-3-Carboxy-2-(3-hydroxy-3-methyl-1-oxobutoxy)-N,N,N-trimethyl-1-propanaminium inner salt
• 実験式（ヒル表記法）: C₁₂H₂₃NO₅
• CAS番号: 99159-87-2
• 分子量: 261.31
• Beilstein: 10549761
• MDL番号: MFCD32061910
• UNSPSCコード: 41116107
• NACRES: NA.24

特性

• グレード: analytical standard
• 品質水準: 100
• アッセイ: ≥90.0% (HPLC)
• 光学活性: [α]/D -17.0±2.0°, c = 0.1 in H₂O
• シェルフライフ: limited shelf life, expiry date on the label
• 不純物: ≤15% water (calc. on elemental analysis)
• アプリケーション: clinical testing
• フォーマット: neat
• 保管温度: 2-8°C
• SMILES記法: 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

詳細

生物化学的／生理学的作用

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.

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安全性情報

• 保管分類コード: 11 - Combustible Solids
• WGK: WGK 3
• 引火点(°F): Not applicable
• 引火点(℃): Not applicable