Skip to main content

Gangliosides: Synthesis and Function in Nervous Tissues

  • Reference work entry
  • First Online:
Glycoscience: Biology and Medicine

Abstract

Gangliosides, sialic acid-containing glycosphingolipids, have been considered to be involved in the development and functions of the nervous system. Recent progress in the genetic analysis of gangliosides in cultured cells and experimental animals revealed their roles in the maintenance of integrity of nervous tissues and neuroregeneration. The fact that ganglioside-deficient mice exhibited milder abnormalities than expected suggests that compensatory actions of remaining glycolipids might be present, and complex knockout of multiple glycosyltransferase genes might be useful to clarify essential roles of gangliosides.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 899.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 549.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  • Fukumoto S, Mutoh T, Hasegawa T, Miyazaki H, Okada M, Goto G et al (2000) GD3 synthase gene expression in PC12 cells results in the continuous activation of TrkA and ERK1/2 and enhanced proliferation. J Biol Chem 275:5832–5838

    Article  CAS  PubMed  Google Scholar 

  • Furukawa K, Furukawa K (2008) Sialyltransferases and other enzymes involved in the biosynthesis of gangliosides. In: Taniguchi N, Suzuki A, Ito Y, Narimatsu H, Kawasaki T, Hase S (eds) Experimental glycoscience, glycobiology. Springer, Tokyo, pp 56–58

    Chapter  Google Scholar 

  • Furukawa K, Tokuda N, Okuda T, Tajima O (2004) Glycosphingolipids in engineered mice: insights into function. Semin Cell Dev Biol 15:389–396

    Article  CAS  PubMed  Google Scholar 

  • Furukawa K, Tajima O, Okuda T, Tokuda N, Furukawa K (2007) Knockout mice and glycolipids. In: Kamerling JP, Boons GJ, Lee YC, Suzuki A, Taniguchi N, Voragen AGJ (eds) Comprehensive glycoscience. From chemistry to systems biology. Elsevier, Oxford, pp 149–157

    Chapter  Google Scholar 

  • Furukawa K, Ohmi Y, Kondo Y, Ohkawa Y, Hashimoto N, Tajima O, Furukawa K (2014) The role of glycosphingolipids in lipid rafts: lessons from knockout mice. In: Scilence D (ed) Lipid rafts. Properties, controversies and roles in signal transduction, Cell biology research progress. Nova Biomedical, New York, pp 1–20

    Google Scholar 

  • Haraguchi M, Yamashiro S, Yamamoto A, Furukawa K, Takamiya K, Lloyd KO et al (1994) Isolation of GD3 synthase gene by expression cloning of GM3 alpha-2,8-sialyltransferase cDNA using anti-GD2 monoclonal antibody. Proc Natl Acad Sci U S A 91:10455–10459

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Inoue M, Fujii Y, Furukawa K, Okada M, Okumura K, Hayakawa T et al (2002) Refractory skin injury in complex knock-out mice expressing only the GM3 ganglioside. J Biol Chem 277:29881–29888

    Article  CAS  PubMed  Google Scholar 

  • Jennemann R, Sandhoff R, Wang S, Kiss E, Gretz N, Zuliani C et al (2005) Cell-specific deletion of glucosylceramide synthase in brain leads to severe neural defects after birth. Proc Natl Acad Sci U S A 102:12459–12464

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Lloyd KO, Furukawa K (1997) Biosynthesis and functions of gangliosides: recent advances. Glycoconj J 15:627–636

    Article  Google Scholar 

  • Ohmi Y, Tajima O, Ohkawa Y, Mori A, Sugiura Y, Furukawa K (2009) Gangliosides play pivotal roles in the regulation of complement systems and in the maintenance of integrity in nerve tissues. Proc Natl Acad Sci U S A 106:22405–22410

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Ohmi Y, Tajima O, Ohkawa Y, Yamauchi Y, Sugiura Y, Furukawa K (2011) Gangliosides are essential in the protection of inflammation and neurodegeneration via maintenance of lipid rafts: elucidation by a series of ganglioside-deficient mutant mice. J Neurochem 116:926–935

    Article  CAS  PubMed  Google Scholar 

  • Ohmi Y, Ohkawa Y, Yamauchi Y, Tajima O, Furukawa K (2012) Essential roles of gangliosides in the formation and maintenance of membrane microdomains in brain tissues. Neurochem Res 37:1185–1191

    Article  CAS  PubMed  Google Scholar 

  • Ohmi Y, Ohkawa Y, Tajima O, Sugiura Y, Furukawa K, Furukawa K (2014) Ganglioside deficiency causes inflammation and neurodegeneration via the activation of complement system in the spinal cord. J Neuroinflammation 11:61. doi:10.1186/1742-2094-11-61

    Article  PubMed  PubMed Central  Google Scholar 

  • Okada M, Itoh Mi M, Haraguchi M, Okajima T, Inoue M, Oishi H et al (2002) b-series Ganglioside deficiency exhibits no definite changes in the neurogenesis and the sensitivity to Fas-mediated apoptosis but impairs regeneration of the lesioned hypoglossal nerve. J Biol Chem 277:1633–1636

    Article  CAS  PubMed  Google Scholar 

  • Schengrund CL (1990) The role(s) of gangliosides in neural differentiation and repair: a perspective. Brain Res Bull 24:131–141

    Article  CAS  PubMed  Google Scholar 

  • Simons K, Sampaio JL (2011) Membrane organization and lipid rafts. Cold Spring Harb Perspect Biol 3:a004697

    Article  PubMed  PubMed Central  Google Scholar 

  • Sugiura Y, Furukawa K, Tajima O, Mii S, Honda T (2005) Sensory nerve-dominant nerve degeneration and remodeling in the mutant mice lacking complex gangliosides. Neuroscience 135:1167–1178

    Article  CAS  PubMed  Google Scholar 

  • Takamiya K, Yamamoto A, Furukawa K, Yamashiro S, Shin M, Okada M et al (1996) Mice with disrupted GM2/GD2 synthase gene lack complex gangliosides but exhibit only subtle defects in their nervous system. Proc Natl Acad Sci U S A 93:10662–10667

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Wu G, Xie X, Lu ZH, Ledeen RW (2009) Sodium-calcium exchanger complexed with GM1 ganglioside in nuclear membrane transfers calcium from nucleoplasm to endoplasmic reticulum. Proc Natl Acad Sci U S A 106:10829–10834

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Yamashiro S, Haraguchi M, Furukawa K, Takamiya K, Yamamoto A, Nagata Y et al (1995) Substrate specificity of beta 1,4-N-acetylgalactosaminyltransferase in vitro and in cDNA-transfected cells. GM2/GD2 synthase efficiently generates asialo-GM2 in certain cells. J Biol Chem 270:6149–6155

    Article  CAS  PubMed  Google Scholar 

  • Yamashita T, Wada R, Sasaki T, Deng C, Bierfreund U, Sandhoff K et al (1999) A vital role for glycosphingolipid synthesis during development and differentiation. Proc Natl Acad Sci U S A 96:9142–9147

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Yamashita T, Hashiramoto A, Haluzik M, Mizukami H, Beck S, Norton A et al (2003) Enhanced insulin sensitivity in mice lacking ganglioside GM3. Proc Natl Acad Sci U S A 100:3445–3449

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Yao D, McGonigal R, Barrie JA, Cappell J, Cunningham ME, Meehan G et al (2014) Neuronal expression of GalNAc transferase is sufficient to prevent the age-related neurodegenerative phenotype of complex ganglioside-deficient mice. J Neurosci 34:880–891

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Yu RK, Tsai YT, Ariga T (2012) Functional roles of gangliosides in neurodevelopment: an overview of recent advances. Neurochem Res 37:1230–1244

    Article  CAS  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Koichi Furukawa .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer Japan

About this entry

Cite this entry

Furukawa, K. et al. (2015). Gangliosides: Synthesis and Function in Nervous Tissues. In: Taniguchi, N., Endo, T., Hart, G., Seeberger, P., Wong, CH. (eds) Glycoscience: Biology and Medicine. Springer, Tokyo. https://doi.org/10.1007/978-4-431-54841-6_121

Download citation

Publish with us

Policies and ethics