Wattenberg Binks W
Department of Biochemistry and Molecular Biology, Virginia Commonwealth University School of Medicine, Richmond, VA, 23298, USA.
Adv Biol Regul. 2019 Jan;71:97-103. doi: 10.1016/j.jbior.2018.11.002. Epub 2018 Nov 23.
The myelin sheath, produced by oligodendrocytes in the central nervous system, provides essential electrical insulation to neurons, but also is critical for viability of neurons. Both the protein and lipid composition of this fascinating membrane is unique. Here the focus is on the sphingolipids that are highly abundant in myelin and, in particular, how they are produced. This review discusses how sphingolipid metabolism is regulated. In particular the subcellular localization of lipid metabolic enzymes is discussed and how inter-organelle transport can affect the metabolic routes that sphingolipid precursors take. Understanding the regulation of sphingolipid metabolism in formation of the myelin membrane will have a significant impact on strategies to treat demyelinating diseases.
髓鞘由中枢神经系统中的少突胶质细胞产生，为神经元提供必要的电绝缘，对神经元的存活也至关重要。这种迷人的膜的蛋白质和脂质组成都很独特。本文重点关注髓鞘中高度丰富的鞘脂，特别是它们的产生方式。这篇综述讨论了鞘脂代谢是如何被调控的。特别讨论了脂质代谢酶的亚细胞定位，以及细胞器间运输如何影响鞘脂前体所采取的代谢途径。了解髓鞘膜形成过程中鞘脂代谢的调控，将对治疗脱髓鞘疾病的策略产生重大影响。
