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. 2020 Jul 23;13:118. doi: 10.3389/fnmol.2020.00118

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Copyright © 2020 Arreguin and Colognato.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Cortical dysplasia and NSC dysfunction in Congenital Muscular Dystrophies (CMD). Left: normal cortical development. Right: abnormal cortical development in CMD. 1. Breached BM caused by mutations in DAG1 and GPR56 genes, or abnormal glycosylation by fukutin, FKRP, and LARGE1. 2. Impaired oligodendrogenesis due to loss of LAMA2 and DAG1 expression or function. 3. The detachment of NSCs due to the loss of a functional interaction between laminin α2 and β1 integrins. Inset: receptors mediate interactions between radial glial cell endfeet and with laminin α2: 1, Dystroglycan; 2, Integrin; 3, GPR56 and (possibly) GPR126. Abbreviations: BM, basement membrane; CP, cortical plate; IZ, intermediate zone; MZ/BM, Marginal Zone/Basement Membrane; NSC, neural stem cell; SVZ, subventricular zone; VZ, ventricular zone.