AUTHOR=Ayo-Martin Ane Cristina , Kyrousi Christina , Di Giaimo Rossella , Cappello Silvia 

TITLE=GNG5 Controls the Number of Apical and Basal Progenitors and Alters Neuronal Migration During Cortical Development

JOURNAL=Frontiers in Molecular Biosciences

VOLUME=7

YEAR=2020

URL=https://www.frontiersin.org/journals/molecular-biosciences/articles/10.3389/fmolb.2020.578137

DOI=10.3389/fmolb.2020.578137

ISSN=2296-889X

ABSTRACT=<p>Cortical development is a very complex process in which any temporal or spatial alterations can give rise to a wide range of cortical malformations. Among those malformations, periventricular heterotopia (PH) is characterized by clusters of neurons that do not migrate to the correct place. Cerebral organoids derived from patients with mutations in <italic>DCHS1</italic> and <italic>FAT4</italic>, which have been associated with PH, exhibit higher levels of <italic>GNG5</italic> expression in a patient-specific cluster of neurons. Here we investigate the role of GNG5 during the development of the cerebral cortex in mice and human cerebral organoids. <italic>GNG5</italic>, highly expressed in progenitors and downregulated in neurons, is critical for controlling the number of apical and basal progenitors and neuronal migration. Moreover, forced expression of <italic>GNG5</italic> recapitulates some of the alterations observed upon downregulation of <italic>Dchs1</italic> and <italic>Fat4</italic> in mice and human cerebral organoids derived from <italic>DCHS1</italic> and <italic>FAT4</italic> patients, suggesting a critical role of <italic>GNG5</italic> in cortical development.</p>