Neurochemical Research

, Volume 37, Issue 6, pp 1230–1244

Functional Roles of Gangliosides in Neurodevelopment: An Overview of Recent Advances

Overview

DOI: 10.1007/s11064-012-0744-y

Cite this article as:
Yu, R.K., Tsai, YT. & Ariga, T. Neurochem Res (2012) 37: 1230. doi:10.1007/s11064-012-0744-y

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.

Keywords

Glycosyltransferase Ganglioside synthase Gangliosides Knockout mouse Lipid raft Neurodevelopment Neurological disease 

Abbreviations

GSL

Glycosphingolipid

CNS

Central nervous system

PNS

Peripheral nervous system

GT

Glycosyltransferase

GEM

GSL-enriched microdomain

NMJ

Neuromuscular junction

Tag

Transgenic mouse

WT

Wild-type mouse

AD

Alzheimer’s disease

HD

Hungtinton’s disease

PD

Parkinson’s disease

NSC

Neural stem cell

NPC

Neural progenitor cell

GBS

Guillain-Barré syndrome

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  1. 1.Institute of Molecular Medicine and Genetics and Institute of Neuroscience, Medical College of GeorgiaGeorgia Health Sciences UniversityAugustaUSA

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