Forebrain Eml1 depletion reveals early centrosomal dysfunction causing subcortical heterotopia

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Zaidi, Donia | Chinnappa, Kaviya | Yigit, Berfu Nur | Viola, Valeria | Cifuentes-Diaz, Carmen | Jabali, Ammar | Uzquiano, Ana | Lemesre, Emilie | Perez, Franck | Ladewig, Julia | Ferent, Julien | Ozlu, Nurhan | Francis, Fiona

Edité par CCSD ; Rockefeller University Press -

International audience. Mechanisms leading to abnormal distribution of neural progenitors during cortical development in the context of subcortical heterotopia associated with EML1 mutations remain unknown. Using a forebrain-specific mouse model and mutant human cells, this work innovatively demonstrates that by restoring microtubule function, abnormal progenitor distribution and heterotopic volume are significantly reduced.

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