Post-natal treatment by a blood-brain-barrier permeable calpain inhibitor, SNJ1945 rescued defective function in lissencephaly

نویسندگان

  • Shiori Toba
  • Yasuhisa Tamura
  • Kanako Kumamoto
  • Masami Yamada
  • Keizo Takao
  • Satoko Hattori
  • Tsuyoshi Miyakawa
  • Yosky Kataoka
  • Mitsuyoshi Azuma
  • Kiyoshi Hayasaka
  • Masano Amamoto
  • Keiko Tominaga
  • Anthony Wynshaw-Boris
  • Hideki Wanibuchi
  • Yuichiro Oka
  • Makoto Sato
  • Mitsuhiro Kato
  • Shinji Hirotsune
چکیده

Toward a therapeutic intervention of lissencephaly, we applied a novel calpain inhibitor, SNJ1945. Peri-natal or post-natal treatment with SNJ1945 rescued defective neuronal migration in Lis1⁺/⁻ mice, impaired behavioral performance and improvement of ¹⁸F-FDG uptake. Furthermore, SNJ1945 improved the neural circuit formation and retrograde transport of NFG in Lis1⁺/⁻ mice. Thus, SNJ1945 is a potential drug for the treatment of human lissencephaly patients.

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عنوان ژورنال:

دوره 3  شماره 

صفحات  -

تاریخ انتشار 2013