Barnes-Vélez Joseph A, Aksoy Yasar Fatma Betul, Hu Jian
Department of Cancer Biology, The University of Texas MD Anderson Cancer Center, Houston, TX 77054-1901, USA.
MD Anderson Cancer Center UTHealth Graduate School of Biomedical Science, Houston, TX 77225-0334, USA.
Innovation (Camb). 2022 Dec 7;4(1):100360. doi: 10.1016/j.xinn.2022.100360. eCollection 2023 Jan 30.
Myelin is a specialized cell membrane indispensable for rapid nerve conduction. The high abundance of membrane lipids is one of myelin's salient features that contribute to its unique role as an insulator that electrically isolates nerve fibers across their myelinated surface. The most abundant lipids in myelin include cholesterol, glycosphingolipids, and plasmalogens, each playing critical roles in myelin development as well as function. This review serves to summarize the role of lipid metabolism in myelination and myelin maintenance, as well as the molecular determinants of myelin lipid homeostasis, with an emphasis on findings from genetic models. In addition, the implications of myelin lipid dysmetabolism in human diseases are highlighted in the context of hereditary leukodystrophies and neuropathies as well as acquired disorders such as Alzheimer's disease.
髓磷脂是一种对快速神经传导不可或缺的特殊细胞膜。膜脂的高丰度是髓磷脂的显著特征之一，这有助于其作为绝缘体发挥独特作用，在有髓鞘的表面将神经纤维电隔离。髓磷脂中最丰富的脂质包括胆固醇、糖鞘脂和缩醛磷脂，它们在髓磷脂的发育和功能中均发挥着关键作用。本综述旨在总结脂质代谢在髓鞘形成和髓磷脂维持中的作用，以及髓磷脂脂质稳态的分子决定因素，重点介绍遗传模型的研究结果。此外，在遗传性脑白质营养不良、神经病以及阿尔茨海默病等获得性疾病的背景下，强调了髓磷脂脂质代谢异常在人类疾病中的影响。
