Université de Strasbourg, UMR_S 1118, Fédération de Médecine Translationnelle, CRBS, 1 rue Eugène Boeckel, 67000 Strasbourg, France; INSERM, U1118, Mécanismes Centraux et Périphériques de la Neurodégénérescence, CRBS, 1 rue Eugène Boeckel, 67000 Strasbourg, France.
Spedding Research Solutions SAS, 6 rue Ampere, 78650 Le Vesinet, France.
Semin Cell Dev Biol. 2021 Apr;112:82-91. doi: 10.1016/j.semcdb.2020.10.008. Epub 2020 Nov 5.
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.
鞘脂类是复杂的脂质。它们在神经元中起结构作用，但也参与调节细胞通讯以及神经元的分化和成熟。越来越多的证据表明，鞘脂类代谢失调与肌萎缩侧索硬化症（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，我们概述了鞘脂类在神经系统发育和维持中的作用。我们描述了鞘脂类代谢改变在某些神经退行性疾病发病机制中的意义，主要集中在 ALS 上。最后，我们提供了潜在治疗方法的最新进展，这些方法可用于靶向神经退行性疾病中的鞘脂代谢。
