Ataxia and peripheral nerve hypomyelination in ADAM22-deficient mice

Koji Sagane, Kazuhiro Hayakawa, Junko Kai, Tomoko Hirohashi, Eiki Takahashi, Norimasa Miyamoto, Mitsuhiro Ino, Tohru Oki, Kazuto Yamazaki, Takeshi Nagasu

研究成果: ジャーナルへの寄稿学術誌査読

126 被引用数 (Scopus)

抄録

Background: ADAM22 is a member of the ADAM gene family, but the fact that it is expressed only in the nervous systems makes it unique. ADAM22's sequence similarity to other ADAMs suggests it to be an integrin binder and thus to have a role in cell-cell or cell-matrix interactions. To elucidate the physiological functions of ADAM22, we employed gene targeting to generate ADAM22 knockout mice. Results: ADAM22-deficient mice were produced in a good accordance with the Mendelian ratio and appeared normal at birth. After one week, severe ataxia was observed, and all homozygotes died before weaning, probably due to convulsions. No major histological abnormalities were detected in the cerebral cortex or cerebellum of the homozygous mutants; however, marked hypomyelination of the peripheral nerves was observed. Conclusion: The results of our study demonstrate that ADAM22 is closely involved in the correct functioning of the nervous system. Further analysis of ADAM22 will provide clues to understanding the mechanisms of human diseases such as epileptic seizures and peripheral neuropathy.

本文言語英語
論文番号33
ジャーナルBMC Neuroscience
6
DOI
出版ステータス出版済み - 5月 6 2005
外部発表はい

!!!All Science Journal Classification (ASJC) codes

  • 神経科学(全般)
  • 細胞および分子神経科学

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