Functional roles of gangliosides in neurodevelopment: an overview of recent advances

Neurochem Res. 2012 Jun;37(6):1230-44. doi: 10.1007/s11064-012-0744-y. Epub 2012 Mar 13.

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.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.
  • Review

MeSH terms

  • Alzheimer Disease / physiopathology
  • Animals
  • Brain / growth & development*
  • Disease Models, Animal
  • Epigenesis, Genetic / physiology
  • Gangliosides / biosynthesis
  • Gangliosides / physiology*
  • Gene Expression Regulation, Developmental
  • Glycosyltransferases / genetics
  • Glycosyltransferases / metabolism
  • Guillain-Barre Syndrome / physiopathology
  • Humans
  • Huntington Disease / physiopathology
  • Mice
  • Mice, Knockout
  • Nerve Degeneration / physiopathology
  • Neural Stem Cells / physiology
  • Neurodegenerative Diseases / physiopathology
  • Parkinson Disease / physiopathology

Substances

  • Gangliosides
  • Glycosyltransferases