title: Functional roles of gangliosides in neurodevelopment: an overview of recent advances.
abstract: Gangliosides are sialic acid-containing glycosphingolipids that are most abundant in the nervous system. They are localized primarily in the outer leaflets of plasma membranes and participated in cell-cell recognition, adhesion, and signal transduction and are integral components of cell surface microdomains or lipid rafts along with proteins, sphingomyelin and cholesterol. Ganglioside-rich lipid rafts play an important role in signaling events affecting neural development and the pathogenesis of certain diseases. Disruption of gangloside synthase genes in mice induces developmental defects and neural degeneration. Targeting ganglioside metabolism may represent a novel therapeutic strategy for intervention in certain diseases. In this review, we focus on recent advances on metabolic and functional studies of gangliosides in normal brain development and in certain neurological disorders.
authors.affiliation: Institute of Molecular Medicine and Genetics and Institute of Neuroscience, Medical College of Georgia, Georgia Health Sciences University, 1120 15th Street, Augusta, GA 30912, USA. ryu@georgiahealth.edu
authors.forename: Robert K
authors.initials: RK
authors.lastname: Yu
authors.forename: Yi-Tzang
authors.initials: YT
authors.lastname: Tsai
authors.forename: Toshio
authors.initials: T
authors.lastname: Ariga
mesh_terms: D000544:Alzheimer Disease / Q000503:physiopathology; D000818:Animals; D001921:Brain / Q000254:growth & development*; D004195:Disease Models, Animal; D044127:Epigenesis, Genetic / Q000502:physiology; D005732:Gangliosides / Q000096:biosynthesis / Q000502:physiology*; D018507:Gene Expression Regulation, Developmental; D016695:Glycosyltransferases / Q000235:genetics / Q000378:metabolism; D020275:Guillain-Barre Syndrome / Q000503:physiopathology; D006801:Humans; D006816:Huntington Disease / Q000503:physiopathology; D051379:Mice; D018345:Mice, Knockout; D009410:Nerve Degeneration / Q000503:physiopathology; D058953:Neural Stem Cells / Q000502:physiology; D019636:Neurodegenerative Diseases / Q000503:physiopathology; D010300:Parkinson Disease / Q000503:physiopathology
chemical_list: D005732:Gangliosides; D016695:Glycosyltransferases