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KO Mice of β1,4-N-acetylgalactosaminyltransferase (GM2/GD2 Synthase)

  • Koichi Furukawa
  • Kogo Takamiya
  • Keiko Furukawa

Abstract

Complex gangliosides have been considered to play important roles in the development and differentiation of nervous systems in vertebrates. In order to directly address this issue, we generated knockout mice of beta 1,4-N-acetylgalactosaminyltransferase gene (Nagata et al. 1992) that is responsible for the synthesis of GM2 and GD2 (and GA2), and clearly showed that this enzyme is really critical for the synthesis of all complex gangliosides (Takamiya et al. 1996). Although the null mutant mice showed minor neurological disorders, their brain and nervous tissues were almost normally generated. In contrast, they showed aspermatogenesis (Takamiya et al. 1998) and immuno-dysfunctions. However, the null mutants demonstrated neurodegeneration with aging suggesting roles of complex gangliosides in the maintenance of integrity of nervous systems (Sugiura et al. 2005).

Keywords

Target Vector Purkinje Neuron Chimeric Mouse Null Mutant Mouse Complex Ganglioside 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

  1. Nagata Y, Yamashiro S, Yodoi J, Lloyd KO, Shiku H, Furukawa K (1992) Expression cloning of β1,4 N-acetyl-galacto-saminyltransferase cDNAs that determine the expression of GM2 and GD2 gangliosides. J Biol Chem 267:12082–12089PubMedGoogle Scholar
  2. Sugiura Y, Furukawa K, Tajima O, Mii T, Honda T, and Furukawa K (2005) Sensory nerve-dominant nerve degeneration and remodeling in the mutant mice lacking complex gangliosides. Neuroscience 135:1167–1178PubMedCrossRefGoogle Scholar
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Copyright information

© Springer 2008

Authors and Affiliations

  • Koichi Furukawa
    • 1
  • Kogo Takamiya
    • 1
  • Keiko Furukawa
    • 1
  1. 1.Department of Biochemistry IINagoya University Graduate School of MedicineNagoyaJapan

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