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iScience. 2021 Sep 17;24(10):103140. doi: 10.1016/j.isci.2021.103140. eCollection 2021 Oct 22.

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

iScience

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

Affiliations

  1. Department of Clinical Genetics, Fujita Health University Hospital, 1-98 Dengakugakubo, Kutsukake-chou, Toyoake, Aichi 470-1192, Japan.
  2. Division of Molecular Genetics, Institute for Comprehensive Medical Science, Fujita Health University, Toyoake, Aichi 470-1192, Japan.
  3. Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Hyogo 650-0017, Japan.
  4. Division of Advanced Medical Science, Graduate School of Science, Technology and Innovation, Kobe University, Kobe, Hyogo 650-0017, Japan.
  5. Department of iPS Cell Applications, Graduate School of Medicine, Kobe University, Kobe, Hyogo 650-0017, Japan.
  6. Center for Human Resource Development for Regenerative Medicine, Kobe University Hospital, Kobe, Hyogo 650-0017, Japan.
  7. Department of Chemical Science and Engineering, Graduate School of Engineering, Kobe University, Kobe, Hyogo 657-8501, Japan.
  8. Department of Clinical Application, Center for iPS Cell Research and Application, Kyoto University, Sakyo-ku, Kyoto 606-8507, Japan.
  9. Department of Pediatrics, Kyoto University Graduate School of Medicine, Sakyo-ku, Kyoto 606-8507, Japan.
  10. Department of Cellular Function Analysis, Research Promotion and Support Headquarters, Fujita Health University, Toyoake, Aichi 470-1192, Japan.
  11. Asubio Pharma Co., Ltd., Kobe, Hyogo 650-0047, Japan.
  12. Innovative and Clinical Research Promotion Center, Gifu University Hospital, Yanagido, Gifu 501-1194, Japan.
  13. Laboratory for Cell Asymmetry, RIKEN Center for Developmental Biology, Kobe, Hyogo 650-0047, Japan.
  14. Department of iPS Cell Applied Medicine, Graduate School of Medicine, Kansai Medical University, Hirakata, Osaka 573-1010, Japan.
  15. Department of Pediatrics, Tokyo Women's Medical University, School of Medicine, Shinjuku-ku, Tokyo 162-8666, Japan.
  16. Department of Personalized Cancer Immunotherapy, Mie University Graduate School of Medicine, Tsu, Mie 514-8507, Japan.
  17. Department of Neurobiology, David Geffen School of Medicine at the University of California, Los Angeles (UCLA), Los Angeles, CA 90095, USA.
  18. Eli and Edythe Broad Center of Regenerative Medicine and Stem Cell Research, UCLA, Los Angeles, CA, USA.
  19. Intellectual and Developmental Disabilities Research Center, UCLA, Los Angeles, CA 90095, USA.
  20. Department of Neurology, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, 113-8655, Japan.
  21. Department of Anatomy and Neurobiology, School of Medicine, University of California, Irvine, Irvine, CA 92697, USA.
  22. Sue & Bill Gross Stem Cell Research Center, School of Medicine, University of California, Irvine, Irvine, CA 92697, USA.

PMID: 34632335 PMCID: PMC8487058 DOI: 10.1016/j.isci.2021.103140

Abstract

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)

© 2021 The Author(s).

Keywords: Neuroscience; Pathophysiology; Tissue Engineering

Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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