Department of Neurology, Richard T. Johnson Division of Neuroimmunology and Neurological Infections, The Johns Hopkins University School of Medicine, Meyer 6-109, 600 North Wolfe Street, Baltimore, MD 21287, USA.
Neuromolecular Med. 2010 Dec;12(4):301-5. doi: 10.1007/s12017-010-8135-5. Epub 2010 Aug 25.
Although the brain contains a high content of sphingolipids, we know relatively little about the roles that sphingolipids play in regulating neural functions. Once regarded only for their structural roles in maintaining the integrity of cellular and sub-cellular compartments, it is now apparent that many sphingolipid species are biologically active and play important roles in regulating signaling events. Recent technological and scientific advances are rapidly increasing our knowledge of the roles that sphingolipids play in regulating normal neural activity. Likewise, we are beginning to understand how perturbations in sphingolipid metabolism contribute to the pathogenesis of a variety of neurodegenerative conditions. In this special issue of NeuroMolecular Medicine, we present a series of review articles that summarize new and emerging technologies for the analysis of sphingolipids, sphingolipid metabolic pathways, and how dysfunctions in sphingolipid metabolism contribute to neurodegeneration in lysosomal storage disorders, Alzheimer's disease and Multiple Sclerosis.
尽管大脑中含有高含量的神经鞘脂，但我们对神经鞘脂在调节神经功能方面的作用知之甚少。神经鞘脂曾经仅被认为在维持细胞和亚细胞区室的完整性方面具有结构作用，但现在显然许多神经鞘脂种类具有生物活性，在调节信号事件方面发挥着重要作用。最近的技术和科学进步正在迅速增加我们对神经鞘脂在调节正常神经活动中所起作用的认识。同样，我们也开始了解神经鞘脂代谢紊乱如何导致多种神经退行性疾病的发病机制。在本期《神经分子医学》特刊中，我们呈现了一系列综述文章，总结了用于分析神经鞘脂、神经鞘脂代谢途径以及神经鞘脂代谢紊乱如何导致溶酶体贮积症、阿尔茨海默病和多发性硬化症中神经退行性变的新技术。
