2-methylbutyryl-L-carnitine | 256928-75-3

• 分子结构分类: 有机化合物 - 脂质和类脂质分子 - 脂肪酰基
• 英文名称: 2-methylbutyryl-L-carnitine
• 英文别名: 2-methylbutyryl carnitine；2-Methylbutyrylcarnitine；(3R)-3-(2-methylbutanoyloxy)-4-(trimethylazaniumyl)butanoate
• CAS: 256928-75-3
• 化学式: C₁₂H₂₃NO₄
• 分子量: 245.319
• InChiKey: IHCPDBBYTYJYIL-QVDQXJPCSA-N

计算性质

• 辛醇/水分配系数(LogP)：
  1.8
• 重原子数：
  17
• 可旋转键数：
  7
• 环数：
  0.0
• sp3杂化的碳原子比例：
  0.83
• 拓扑面积：
  66.4
• 氢给体数：
  0
• 氢受体数：
  4

安全信息

• WGK Germany：
  3
• 储存条件：
  | 2-8°C |

制备方法与用途

2-甲基丁酰基肉碱是一种脂肪酸的代谢产物，主要存在于人类和动物的血液与尿液中，通过丙酮酸羧化途径生成。研究发现，在脂肪性肝炎（NASH）患者的血浆中，2-甲基丁酰基肉碱表现出高水平，可以作为诊断代谢性疾病的一个指标。

反应信息

• 作为反应物：
  描述：

  2-methylbutyryl-L-carnitine 在 N,N-二异丙基乙胺 作用下， 以 乙腈 为溶剂， 反应 0.5h， 生成 2-methylbutyryl-L-carnitine oxylactone
  参考文献：
  名称：

  Characterization of acylcarnitines using fast atom bombardment mass spectrometry and gas chromatography/mass spectrometry

  摘要：

  报告了一系列合成酰基肉碱的质谱数据。采用快速原子轰击离子化技术结合高能碰撞激发和B/E连扫的直接质谱方法。数据显示，可以使用该技术区分异构化合物。报告了以酰氧乳酸酯衍生物形式的酰基肉碱的气相色谱/质谱分析数据。使用了电子轰击和化学电离两种模式，这使得各种化合物的特征和鉴定变得明确无误。鉴于最近酰基肉碱分析活动的显著增加，详细报告的质谱和气相色谱数据可能对参考目的有所帮助。

  DOI：

  10.1002/jms.1190300112
• 作为产物：
  描述：

  2-甲基丁酸 在 氯化亚砜 、 三氯乙酸 作用下， 反应 22.0h， 生成 2-methylbutyryl-L-carnitine
  参考文献：
  名称：

  An LC-MS/MS method to quantify acylcarnitine species including isomeric and odd-numbered forms in plasma and tissues

  摘要：

  Acylcarnitines are intermediates of fatty acid and amino acid oxidation found in tissues and body fluids. They are important diagnostic markers for inherited diseases of peroxisomal and mitochondrial oxidation processes and were recently described as biomarkers of complex diseases like the metabolic syndrome. Quantification of acylcarnitine species can become challenging because various species occur as isomers and/or have very low concentrations. Here we describe a new LC-MS/MS method for quantification of 56 acylcarnitine species with acyl-chain lengths from C2 to C18. Our method includes amino acid-derived positional isomers, like methacrylyl-carnitine (2-M-C3:1-CN) and crotonyl-carnitine (C4:1-CN), and odd-numbered carbon species, like pentadecanoyl-carnitine (C15:0-CN) and heptadecanoyl-carnitine (C17:0-CN), occurring at very low concentrations in plasma and tissues. Method validation in plasma and liver samples showed high sensitivity and excellent accuracy and precision. In an application to samples from streptozotocin-treated diabetic mice, we identified significantly increased concentrations of acylcarnitines derived from branched-chain amino acid degradation and of odd-numbered straight-chain species, recently proposed as potential biomarkers for the metabolic syndrome. In conclusion, the LC-MS/MS method presented here allows robust quantification of isomeric acylcarnitine species and extends the palette of acylcarnitines with diagnostic potential derived from fatty acid and amino acid metabolism.

  DOI：

  10.1194/jlr.d061721

文献信息

• Use of L-carnitine derivatives for the manufacture of a medicament for the therapeutical treatment of peripheral neuropathies
  申请人：Sigma-Tau Industrie Farmaceutiche Riunite S.p.A.

  公开号：EP0376899A2

  公开（公告）日：1990-07-04

  The use is disclosed of compounds of general formula wherein: Y is hydrogen, methyl or amino, and R is an unsubstituted or substituted alkyl group selected from methyl, ethyl, 1-methylpropyl, isobutyl, isopropyl, mercaptomethyl and 3-guanidinopropyl and the pharmacologically acceptable salts thereof, for treating degenerative alterations of the nervous system. The compounds can be administered orally or parenterally.

  公开了通式如下的化合物的用途 其中 Y 是氢、甲基或氨基，以及 R 是选自甲基、乙基、1-甲基丙基、异丁基、异丙基、巯基甲基和 3-胍基丙基的未取代或取代的烷基。 及其药理上可接受的盐类，用于治疗神经系统的退行性改变。这些化合物可以口服或肠外给药。
• L-carnitine derivatives as therapeutical agents for treating myopathies and neuronal degeneration and for inhibiting proteolysis
  申请人：Sigma-Tau Industrie Farmaceutiche Riunite S.p.A.

  公开号：EP0516594A1

  公开（公告）日：1992-12-02

  The use is disclosed of compounds of general formula    wherein: Y is hydrogen or methyl and R is an unsubstituted or substituted alkyl group selected from methyl, ethyl and isopropyl, and the pharmacologically acceptable salts thereof, for treating myopathies, neuronal degeneration and some pathologies affecting the liver, skeletal muscles and myocardium. The compounds can be administered orally or parenterally.

  公开了通式化合物的用途 其中 Y 是氢或甲基 R 是选自甲基、乙基和异丙基的未取代或取代的烷基、 及其药理学上可接受的盐类，用于治疗肌病、神经元变性以及影响肝脏、骨骼肌和心肌的某些病变。 这些化合物可以口服或肠外给药。
• Differentiation of isomeric acyl carnitines using tandem mass spectrometry
  作者：Simon J. Gaskell、Christian. Guenat、David S. Millington、David A. Maltby、Charles R. Roe

  DOI：10.1021/ac00126a048

  日期：1986.11.1
• Substrate specificity of human carnitine acetyltransferase: Implications for fatty acid and branched-chain amino acid metabolism
  作者：Sara Violante、Lodewijk IJlst、Jos Ruiter、Janet Koster、Henk van Lenthe、Marinus Duran、Isabel Tavares de Almeida、Ronald J.A. Wanders、Sander M. Houten、Fátima V. Ventura

  DOI：10.1016/j.bbadis.2013.02.012

  日期：2013.6

  Carnitine acyltransferases catalyze the reversible conversion of acyl-CoAs into acylcarnitine esters. This family includes the mitochondrial enzymes carnitine palmitoyltransferase 2 (CPT2) and carnitine acetyltransferase (CrAT). CPT2 is part of the carnitine shuttle that is necessary to import fatty acids into mitochondria and catalyzes the conversion of acylcarnitines into acyl-CoAs. In addition, when mitochondrial fatty acid P-oxidation is impaired, CPT2 is able to catalyze the reverse reaction and converts accumulating long- and medium-chain acyl-CoAs into acylcarnitines for export from the matrix to the cytosol. However, CPT2 is inactive with short-chain acyl-CoAs and intermediates of the branched-chain amino acid oxidation pathway (BCAAO). In order to explore the origin of short-chain and branched-chain acylcarnitines that may accumulate in various organic acidemias, we performed substrate specificity studies using purified recombinant human CrAT. Various saturated, unsaturated and branched-chain acyl-CoA esters were tested and the synthesized acylcarnitines were quantified by ESI-MS/MS. We show that CrAT converts short- and medium-chain acyl-CoAs (C2 to C10-00A), whereas no activity was observed with long-chain species. Trans-2-enoyl-00A intermediates were found to be poor substrates for this enzyme. Furthermore, CrAT turned out to be active towards some but not all the BCAAO intermediates tested and no activity was found with dicarboxylic acyl-CoA esters. This suggests the existence of another enzyme able to handle the acyl-CoAs that are not substrates for CrAT and CPT2, but for which the corresponding acylcarnitines are well recognized as diagnostic markers in inborn errors of metabolism. (C) 2013 Elsevier B.V. All rights reserved.
• US5227518A
  申请人：——

  公开号：US5227518A

  公开（公告）日：1993-07-13