Magnesium-d-fructose-1.6-diphosphat | 3671-39-4

• 分子结构分类: 有机化合物 - 有机氧化合物
• 英文名称: Magnesium-d-fructose-1.6-diphosphat
• CAS: 3671-39-4；132418-66-7
• 化学式: C₆H₁₀O₁₂P₂*2Mg
• 分子量: 384.696
• InChiKey: CMJTUCMLIAQNJS-BAOOBMCLSA-J

计算性质

• 辛醇/水分配系数(LogP)：
  -6.05
• 重原子数：
  21.0
• 可旋转键数：
  9.0
• 环数：
  0.0
• sp3杂化的碳原子比例：
  0.83
• 拓扑面积：
  222.6
• 氢给体数：
  3.0
• 氢受体数：
  12.0

反应信息

• 作为反应物：
  描述：

  Magnesium-d-fructose-1.6-diphosphat 生成 丙酮醛
  参考文献：
  名称：

  Widmann, Biochemische Zeitschrift, 1929, vol. 216, p. 476

  摘要：

  DOI：

文献信息

• Infantile Dilated X-Linked Cardiomyopathy, G4.5 Mutations, Altered Lipids, and Ultrastructural Malformations of Mitochondria in Heart, Liver, and Skeletal Muscle
  作者：John J Bissler、Monica Tsoras、Harald H H Göring、Peter Hug、Gail Chuck、Esther Tombragel、Catherine McGraw、James Schlotman、Michael A Ralston、George Hug

  DOI：10.1038/labinvest.3780427

  日期：2002.3

  Mutations in the Xq28 gene G4.5 lead to dilated cardiomyopathy (DCM). Differential splicing of G4.5 results in a family of proteins called "tafazzins" with homology to acyltransferases. These enzymes assemble fatty acids into membrane lipids. We sequenced G4.5 in two kindreds with X-linked DCM and in two unrelated men, one with idiopathic DCM and the other with DCM of arrhythmogenic right ventricular dysplasia. We examined the ultrastructure of heart, liver, and muscle biopsy specimens in these three DCM types; we used gas chromatography to compare fatty acid composition in heart, liver, and muscle autopsy specimens of two patients of kindred 1 with that of controls. In X-linked DCM, G4.5 had a stop codon (E188X), a nonsense mutation, in kindred 1 and an amino acid substitution (G240R), a missense mutation, in kindred 2. In the two men with isolated DCM, G4.5 was not mutated. Ultrastructural mitochondrial malformations were present in the biopsy tissues of the patients with DCM. Cardiac biopsy specimens of both kindreds with X-linked DCM exhibited greatly enlarged mitochondria with large bundles of stacked, compacted, disarrayed cristae that differed from those of the two types of isolated DCM. Autopsy tissue of patients with X-linked DCM had decreased unsaturated and increased saturated fatty acid concentrations. Seven of 13 published G4.5 missense mutations, including the one presented here, occur in acyltransferase motifs. Impaired acyltransferase function could result in increased fatty acid saturation that would decrease membrane fluidity. Mitochondrial membrane proliferation may be an attempt to compensate for impaired function of acyltransferase. Cardiac ultrastructure separates X-linked DCM with G4.5 mutations from the two types of isolated DCM without G4.5 mutations. Electron microscopy of promptly fixed myocardial biopsy specimens has a role in defining the differential diagnosis of DCM. Mutational analysis of the G4.5 gene also serves this purpose.
• Kobel; Scheuer, Biochemische Zeitschrift, 1929, vol. 216, p. 221
  作者：Kobel、Scheuer

  DOI：——

  日期：——
• Neuberg; Kobel, Biochemische Zeitschrift, 1929, vol. 210, p. 475
  作者：Neuberg、Kobel

  DOI：——

  日期：——
• Fromageot, Biochemische Zeitschrift, 1929, vol. 216, p. 472
  作者：Fromageot

  DOI：——

  日期：——
• Vogt, Biochemische Zeitschrift, 1929, vol. 211, p. 17
  作者：Vogt

  DOI：——

  日期：——