Mice with disrupted GM2/GD2 synthase gene lack complex gangliosides but exhibit only subtle defects in their nervous system

Kogo Takamiya, Akihito Yamamoto, Keiko Furukawa, Shuji Yamashiro, Masashi Shin, Masahiko Okada, Satoshi Fukumoto, Masashi Haraguchi, Naoki Takeda, Koichi Fujimura, Mihoko Sakae, Masao Kishikawa, Hiroshi Shiku, Koichi Furukawa, Shinichi Aizawa

Research output: Contribution to journalArticlepeer-review

316 Citations (Scopus)

Abstract

Gangliosides, sialic acid-containing glycosphingolipids, are abundant in the vertebrate (mammalian) nervous system. Their composition is spatially and developmentally regulated, and gangliosides have been widely believed to play essential roles in establishment of the nervous system, especially in neuritogenesis and synaptogenesis. However, this has never been tested directly. Here we report the generation of mice with a disrupted β1,4-N- acetylgalactosaminyltransferase (GM2/GD2 synthase; EC 2,4.1.92) gone. The mice lacked all complex gangliosides. Nevertheless, they did not show any major histological defects in their nervous systems or in gross behavior. Just a slight reduction in the neural conduction velocity from the tibial nerve to the somatosensory cortex, but not to the lumbar spine, was detected. These findings suggest that complex gangliosides are required in neuronal functions but not in the morphogenesis and organogenesis of the brain. The higher levels of GM3 and GD3 expressed in the brains of these mutant mice may be able to compensate for the lack of complex gangliosides.

Original languageEnglish
Pages (from-to)10662-10667
Number of pages6
JournalProceedings of the National Academy of Sciences of the United States of America
Volume93
Issue number20
DOIs
Publication statusPublished - 1996 Oct 1

Keywords

  • development
  • gene knock-out
  • β1, 4GalNAc transferase

ASJC Scopus subject areas

  • General

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