Dorninger Fabian, Forss-Petter Sonja, Berger Johannes
Department of Pathobiology of the Nervous System, Center for Brain Research, Medical University of Vienna, Austria.
FEBS Lett. 2017 Sep;591(18):2761-2788. doi: 10.1002/1873-3468.12788. Epub 2017 Sep 7.
The emerging diverse roles of ether (phospho)lipids in nervous system development and function in health and disease are currently attracting growing interest. Plasmalogens, a subgroup of ether lipids, are important membrane components involved in vesicle fusion and membrane raft composition. They store polyunsaturated fatty acids and may serve as antioxidants. Ether lipid metabolites act as precursors for the formation of glycosyl-phosphatidyl-inositol anchors; others, like platelet-activating factor, are implicated in signaling functions. Consolidating the available information, we attempt to provide molecular explanations for the dramatic neurological phenotype in ether lipid-deficient human patients and mice by linking individual functional properties of ether lipids with pathological features. Furthermore, recent publications have identified altered ether lipid levels in the context of many acquired neurological disorders including Alzheimer's disease (AD) and autism. Finally, current efforts to restore ether lipids in peroxisomal disorders as well as AD are critically reviewed.
醚（磷酸）脂质在神经系统发育以及健康和疾病状态下的功能中所呈现出的多样化新角色，目前正吸引着越来越多的关注。缩醛磷脂作为醚脂质的一个亚组，是参与囊泡融合和膜筏组成的重要膜成分。它们储存多不饱和脂肪酸并可能充当抗氧化剂。醚脂质代谢产物作为糖基磷脂酰肌醇锚形成的前体；其他一些物质，如血小板活化因子，则与信号传导功能有关。综合现有信息，我们试图通过将醚脂质的个体功能特性与病理特征相联系，为醚脂质缺乏的人类患者和小鼠中出现的显著神经学表型提供分子层面的解释。此外，最近的出版物已确定在包括阿尔茨海默病（AD）和自闭症在内的许多后天性神经疾病中，醚脂质水平发生了改变。最后，对目前在过氧化物酶体疾病以及AD中恢复醚脂质的努力进行了批判性综述。
