Restoration of the defect in radial glial fiber migration and cortical plate organization in a brain organoid model of Fukuyama muscular dystrophy

Summary: Fukuyama congenital muscular dystrophy (FCMD) is a severe, intractable genetic disease that affects the skeletal muscle, eyes, and brain and is attributed to a defect in alpha dystroglycan (αDG) O-mannosyl glycosylation. We previously established disease models of FCMD; however, they did no...

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Main Authors: Mariko Taniguchi-Ikeda, Michiyo Koyanagi-Aoi, Tatsuo Maruyama, Toru Takaori, Akiko Hosoya, Hiroyuki Tezuka, Shotaro Nagase, Takuma Ishihara, Taisuke Kadoshima, Keiko Muguruma, Keiko Ishigaki, Hidetoshi Sakurai, Akira Mizoguchi, Bennett G. Novitch, Tatsushi Toda, Momoko Watanabe, Takashi Aoi
Format: Article
Language:English
Published: Elsevier 2021-10-01
Series:iScience
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Online Access:http://www.sciencedirect.com/science/article/pii/S2589004221011081