Plasmalogens (Pls) are a class of membrane phospholipids which serve a number of essential biological functions. Deficiency of Pls is associated with common disorders such as Alzheimer's disease or ischemic heart disease. A complete lack of Pls due to genetically determined defective biosynthesis gives rise to rhizomelic chondrodysplasia punctata (RCDP), characterized by a number of severe disabling pathologic features and death in early childhood. Frequent cardiac manifestations of RCDP include septal defects, mitral valve prolapse, and patent ductus arteriosus. In a mouse model of RCDP, reduced nerve conduction velocity was partially rescued by dietary oral supplementation of the Pls precursor batyl alcohol (BA). Here, we examine the impact of Pls deficiency on cardiac impulse conduction in a similar mouse model (Gnpat KO). In‐vivo electrocardiographic recordings showed that the duration of the QRS complex was significantly longer in Gnpat KO mice than in age‐ and sex‐matched wild‐type animals, indicative of reduced cardiac conduction velocity. Oral supplementation of BA for 2 months resulted in normalization of cardiac Pls levels and of the QRS duration in Gnpat KO mice but not in untreated animals. BA treatment had no effect on the QRS duration in age‐matched wild‐type mice. These data suggest that Pls deficiency is associated with increased ventricular conduction time which can be rescued by oral BA supplementation.

中文翻译：

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口服丁醇补充剂可挽救醚磷脂缺陷小鼠的心脏传导降低。

缩醛磷脂 (Pls) 是一类具有许多基本生物学功能的膜磷脂。Pls 缺乏与阿尔茨海默病或缺血性心脏病等常见疾病有关。由于基因决定的生物合成缺陷而完全缺乏 Pls 会导致根状软骨发育不良（RCDP），其特征是儿童早期出现许多严重的致残病理特征和死亡。RCDP 的常见心脏表现包括间隔缺损、二尖瓣脱垂和动脉导管未闭。在 RCDP 的小鼠模型中，通过膳食口服补充 Pls 前体鲸蜡醇 (BA) 可以部分挽救降低的神经传导速度。在这里，我们检查了 Pls 缺乏对类似小鼠模型（Gnpat高）。体内心电图记录显示， Gnpat KO 小鼠的 QRS 复合波持续时间明显长于年龄和性别匹配的野生型动物，表明心脏传导速度降低。口服补充 BA 2 个月导致Gnpat KO 小鼠的心脏 Pls 水平和 QRS 持续时间正常化，但未治疗的动物没有。BA 治疗对年龄匹配的野生型小鼠的 QRS 持续时间没有影响。这些数据表明，Pls 缺乏与增加的心室传导时间有关，这可以通过口服 BA 补充剂来挽救。