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

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 (18)F-FDG uptake. Furthermore, SNJ1945 improve...

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Detalles Bibliográficos
Autores principales: Toba, Shiori, Tamura, Yasuhisa, Kumamoto, Kanako, Yamada, Masami, Takao, Keizo, Hattori, Satoko, Miyakawa, Tsuyoshi, Kataoka, Yosky, Azuma, Mitsuyoshi, Hayasaka, Kiyoshi, Amamoto, Masano, Tominaga, Keiko, Wynshaw-Boris, Anthony, Wanibuchi, Hideki, Oka, Yuichiro, Sato, Makoto, Kato, Mitsuhiro, Hirotsune, Shinji
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3565454/
https://www.ncbi.nlm.nih.gov/pubmed/23390575
http://dx.doi.org/10.1038/srep01224
Descripción
Sumario: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 (18)F-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.