Sphingolipids metabolism alteration in the central nervous system: Amyotrophic lateral sclerosis (ALS) and other neurodegenerative diseases. (April 2021)

• Record Type: Journal Article
• Authors: Bouscary, Alexandra; Quessada, Cyril; René, Frédérique; Spedding, Michael; Turner, Bradley J.; Henriques, Alexandre; Ngo, Shyuan T.; Loeffler, Jean-Philippe
• Is Part Of: Seminars in cell & developmental biology. Volume 112(2021)
• Page Start: 82
• Page End: 91
• Publication Date: 2021-04
• Subjects: ALS amyotrophic lateral sclerosis -- ER endoplasmic reticulum -- UGT8 galactosyltransferase -- FAPP2 four-phosphate adaptator protein 2 -- SM sphingomyelin -- CNS central nervous system -- UGCG UDP-glucose ceramide glycosyltransferase -- MAGs myelin-associated glycoproteins -- SMase sphingomyelinase -- Trk Tropomyosin receptor kinase -- AD Alzheimer's disease -- PD Parkinson's disease -- GBA β-Glucocerebrosidase -- S1P sphingosine-1-phosphate -- BBB blood-brain barrier; Amyotrophic lateral sclerosis -- Sphingolipids -- Glucosylceramide -- Glucocerebrosidase -- Therapeutic target -- Ambroxol; Cytology -- Periodicals; Developmental biology -- Periodicals; 571.6
• DOI: 10.1016/j.semcdb.2020.10.008
• Languages: English
• ISSNs: 1084-9521

Abstract

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.