Morphological and functional aspects of progenitors perturbed in cortical malformations

Frontiers in Cellular Neuroscience
Sara Bizzotto, Fiona Francis

Abstract

In this review, we discuss molecular and cellular mechanisms important for the function of neuronal progenitors during development, revealed by their perturbation in different cortical malformations. We focus on a class of neuronal progenitors, radial glial cells (RGCs), which are renowned for their unique morphological and behavioral characteristics, constituting a key element during the development of the mammalian cerebral cortex. We describe how the particular morphology of these cells is related to their roles in the orchestration of cortical development and their influence on other progenitor types and post-mitotic neurons. Important for disease mechanisms, we overview what is currently known about RGC cellular components, cytoskeletal mechanisms, signaling pathways and cell cycle characteristics, focusing on how defects lead to abnormal development and cortical malformation phenotypes. The multiple recent entry points from human genetics and animal models are contributing to our understanding of this important cell type. Combining data from phenotypes in the mouse reveals molecules which potentially act in common pathways. Going beyond this, we discuss future directions that may provide new data in this expanding area.

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Citations

Aug 25, 2015·Neurobiology of Disease·Melissa A StoufferFiona Francis
Mar 23, 2017·Scientific Reports·Marine H LaporteRémy Sadoul
Feb 21, 2018·Cell Cycle·Fiona Francis, Richard Belvindrah
Mar 24, 2018·Journal of Neurochemistry·Ana UzquianoFiona Francis
Apr 9, 2016·The EMBO Journal·Virginia FernándezVíctor Borrell
Jun 8, 2019·Journal of Anatomy·Stephan C CollinsFiona Francis
May 12, 2017·FEBS Letters·Caroline A JohnsonH Troy Ghashghaei
Sep 5, 2019·Frontiers in Cellular Neuroscience·Maxime PenissonFiona Francis
Apr 11, 2020·Proceedings of the National Academy of Sciences of the United States of America·Katrina C McNeely, Noelle D Dwyer
Nov 13, 2020·Frontiers in Cell and Developmental Biology·Julien FerentFiona Francis
May 18, 2021·Frontiers in Cell and Developmental Biology·Carlotta Gilardi, Nereo Kalebic
Oct 12, 2021·Physiological Reviews·Lucia Del-Valle-Anton, Víctor Borrell

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Methods Mentioned

BETA
exome sequencing
Transgenic
Knockout
glycosylation
GTPase
nuclear translocation
dissect

Software Mentioned

Treacle

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