Sphingolipids are complex lipids. They play a structural role in neurons, but are also involved in regulating cellular communication, and neuronal differentiation and maturation. There is increasing evidence to suggest that dysregulated metabolism of sphingolipids is linked to neurodegenerative processes in amyotrophic lateral sclerosis (ALS), Parkinson's disease and Gaucher's disease. In this review, we provide an overview of the role of sphingolipids in the development and maintenance of the nervous system. We describe the implications of altered metabolism of sphingolipids in the pathophysiology of certain neurodegenerative diseases, with a primary focus on ALS. Finally, we provide an update of potential treatments that could be used to target the metabolism of sphingolipids in neurodegenerative diseases.

鞘脂是复杂的脂质。它们在神经元中发挥结构作用，但也参与调节细胞通讯、神经元分化和成熟。越来越多的证据表明，鞘脂代谢失调与肌萎缩侧索硬化 (ALS)、帕金森病和戈谢病的神经退行性过程有关。在这篇综述中，我们概述了鞘脂在神经系统发育和维持中的作用。我们描述了鞘脂代谢改变在某些神经退行性疾病的病理生理学中的影响，主要关注 ALS。最后，我们提供了可用于靶向神经退行性疾病中鞘脂代谢的潜在治疗方法的更新。