Kunze D, Reichmann G, Egger E, Olthoff D, Döhler K
Eur J Clin Invest. 1975 Nov 21;5(6):471-5. doi: 10.1111/j.1365-2362.1975.tb00479.x.
The fatty acid distribution of the main lipid fractions: triglycerides (TG), phosphatidylcholine (PCh), phosphatidylethanolamine (PE), and sphingomyelin (Sph) of muscle from 6 patients with progressive muscular dystrophy (p.m.d.), Duchenne, 8 to 12 years old was estimated and compared with normal controls of different age. In view of the results of several authors about varied fatty acid distribution in immature muscle a third group comprising samples of neonatal muscle was studied. 1. The fatty acid pattern of the lipid fractions TG, Sph, and PE from muscle of patients with p.m.d. shows no important variation in comparison to normal controls. In contrast to this the fatty acid distribution in PCh is extremely varied: the percentage of 18:2 is decreased and corrrespondingly the content of 18:1 is increased. In view of the high percentage (nearly 10%) in which linoleic acid is substituted by oleic acid in PCh, effects on the plasma membrane are to be expected. 2. The fatty acid pattern in neonatal muscle shows in narly all positions of the fractions TG, Sph, PE, and PCh a different distribution from normal or dystrophic muscle. In view of the most important variation in dystrophic muscle it must be stated that generally 18:2 is decreased. This deficit was replaced by an increase of all other fatty acids (not only at a substitution by 18:1 as given in p.m.d.). Therefore the diminished content of linoleic acid in PCh of neonatal and dystrophic muscle cannot be interpreted as expression of a corresponding or similar lipid metabolism in both tissues. The results were seen as signs of significant qualitative alterations especially of PCh in p.m.d. They were discussed as proof of our thesis that the basic defect in p.m.d. concerns the specific acylation of PCh with linoleic acid.
对6例8至12岁杜兴氏进行性肌营养不良（p.m.d.）患者肌肉中主要脂质成分，即甘油三酯（TG）、磷脂酰胆碱（PCh）、磷脂酰乙醇胺（PE）和鞘磷脂（Sph）的脂肪酸分布进行了估算，并与不同年龄的正常对照组进行了比较。鉴于几位作者关于未成熟肌肉中脂肪酸分布各异的研究结果，对包含新生儿肌肉样本的第三组进行了研究。1. p.m.d.患者肌肉中TG、Sph和PE脂质成分的脂肪酸模式与正常对照组相比无重要差异。与此相反，PCh中的脂肪酸分布差异极大：18:2的百分比降低，相应地18:1的含量增加。鉴于PCh中亚油酸被油酸替代的比例较高（近10%），预计会对质膜产生影响。2. 新生儿肌肉中的脂肪酸模式在TG、Sph、PE和PCh成分的几乎所有位置都显示出与正常或营养不良肌肉不同的分布。鉴于营养不良肌肉中最重要的差异，必须指出一般18:2会减少。这种不足被所有其他脂肪酸的增加所取代（不仅如p.m.d.中那样被18:1取代）。因此，新生儿和营养不良肌肉中PCh中亚油酸含量的减少不能被解释为两种组织中相应或相似脂质代谢的表现。这些结果被视为p.m.d.中特别是PCh发生显著定性改变的迹象。它们被作为我们论点的证据进行了讨论，即p.m.d.的基本缺陷涉及PCh与亚油酸的特定酰化作用。
