Williams K A, Deber C M
Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada.
Crit Rev Clin Lab Sci. 1993;30(1):29-64. doi: 10.3109/10408369309084665.
Multiple sclerosis (MS) is characterized by the active degradation of central nervous system myelin, a multilamellar membrane system that insulates nerve axons. MS arises from complex interactions between genetic, immunological, infective, and biochemical mechanisms. Although the circumstances of MS etiology remain hypothetical, one persistent theme involves immune system recognition of myelin-specific antigens derived from myelin basic protein, the most abundant extrinsic myelin membrane protein, and/or another equally suitable myelin protein or lipid. Knowledge of the biochemical and physical-chemical properties of myelin proteins, and lipids, particularly their composition, organization, structure, and accessibility with respect to the compacted myelin multilayers, thus becomes central to understanding how and why these antigens become selected during the development of MS. This article focuses on the current understanding of the molecular basis of MS as it may relate to the protein and lipid components of myelin, which dictate myelin morphology on the basis of protein-lipid and lipid-lipid interactions, and the relationship, if any, between the protein/lipid components and the destruction of myelin in pathological situations.
多发性硬化症（MS）的特征是中枢神经系统髓鞘的主动降解，髓鞘是一种包裹神经轴突的多层膜系统。MS源于遗传、免疫、感染和生化机制之间的复杂相互作用。尽管MS病因的具体情况仍属假设，但一个持续存在的主题涉及免疫系统对源自髓鞘碱性蛋白（最丰富的外在髓鞘膜蛋白）和/或另一种同样合适的髓鞘蛋白或脂质的髓鞘特异性抗原的识别。因此，了解髓鞘蛋白和脂质的生化及物理化学性质，特别是它们的组成、组织、结构以及相对于紧密的髓鞘多层结构的可及性，对于理解这些抗原在MS发展过程中如何以及为何被选择至关重要。本文重点关注对MS分子基础的当前理解，因为它可能与髓鞘的蛋白质和脂质成分相关，这些成分基于蛋白质-脂质和脂质-脂质相互作用决定髓鞘形态，以及蛋白质/脂质成分与病理情况下髓鞘破坏之间的关系（如果存在的话）。
