Department of Biology, Georgia State University, Atlanta, GA 30303, USA.
Pediatric Neurology Unit, Department of Pediatrics, Navarra Health Service Hospital, Irunlarrea 4, 310620 Pamplona, Spain; Navarrabiomed-Miguel Servet Research Foundation, Pamplona, Spain.
Neurobiol Dis. 2020 Sep;143:105014. doi: 10.1016/j.nbd.2020.105014. Epub 2020 Jul 9.
In recent years, research on sphingolipids, particularly ceramides, has attracted increased attention, revealing the important roles and many functions of these molecules in several human neurological disorders. The nervous system is enriched with important classes of sphingolipids, e.g., ceramide and its derivatives, which compose the major portion of this group, particularly in the form of myelin. Ceramides have also emerged as important nodes for lipid signalling, both inside the cell and between cells. Until recently, knowledge about ceramides in the nervous system was limited, but currently, multiple links between ceramide signalling and neurological diseases have been reported. Alterations in the regulation of ceramide pathobiology have been shown to influence the risk of developing neurometabolic diseases. Thus, these molecules are critically important in the maintenance and development of the nervous system and are culprits or major contributors to the development of brain disorders, either inherited or multifactorial. In the present review, we highlight the critical role of ceramide signalling in several different neurological disorders as well as the effects of their perturbations and discuss how this emerging class of bioactive sphingolipids has attracted interest in the field of neurological diseases.
近年来，对神经鞘脂类，特别是神经酰胺的研究受到了越来越多的关注，揭示了这些分子在多种人类神经紊乱中的重要作用和多种功能。神经鞘脂类富含重要的神经鞘脂类，例如神经酰胺及其衍生物，它们构成了该类物质的主要部分，特别是在髓鞘中。神经酰胺也已成为细胞内和细胞间脂质信号传递的重要节点。直到最近，人们对神经系统中神经酰胺的了解还很有限，但目前，已有多种报道称神经酰胺信号与神经疾病之间存在联系。神经酰胺病理生物学调节的改变已被证明会影响神经代谢疾病的发病风险。因此，这些分子在神经系统的维持和发育中至关重要，是脑疾病发展的罪魁祸首或主要贡献者，无论是遗传性的还是多因素的。在本综述中，我们重点介绍了神经酰胺信号在几种不同神经紊乱中的关键作用，以及它们的干扰的影响，并讨论了这一新兴的生物活性神经鞘脂类如何引起神经疾病领域的关注。
