Ceramide signalling in inherited and multifactorial brain metabolic diseases.

Autor: Pant DC; Department of Biology, Georgia State University, Atlanta, GA 30303, USA., Aguilera-Albesa S; Pediatric Neurology Unit, Department of Pediatrics, Navarra Health Service Hospital, Irunlarrea 4, 310620 Pamplona, Spain; Navarrabiomed-Miguel Servet Research Foundation, Pamplona, Spain., Pujol A; Neurometabolic Diseases Laboratory, IDIBELL, Hospital Duran i Reynals, Gran Via 199, 08908, L'Hospitalet de Llobregat, Barcelona, Spain; Catalan Institution of Research and Advanced Studies (ICREA), Barcelona, Catalonia, Spain; Center for Biomedical Research on Rare Diseases (CIBERER), ISCIII, Madrid, Spain. Electronic address: apujol@idibell.cat.
Jazyk: angličtina
Zdroj: Neurobiology of disease [Neurobiol Dis] 2020 Sep; Vol. 143, pp. 105014. Date of Electronic Publication: 2020 Jul 09.
DOI: 10.1016/j.nbd.2020.105014
Abstrakt: 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.
(Copyright © 2020. Published by Elsevier Inc.)
Databáze: MEDLINE