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Published on: March 24, 2019
Sphingolipids metabolism alteration in the central nervous system: Amyotrophic lateral sclerosis (ALS) and other
Alexandra Bouscary1, Cyril Quessada1, Frédérique René1
1Université 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.
Abstract:
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.
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