Journal
FRONTIERS IN MOLECULAR BIOSCIENCES
Volume 7, Issue -, Pages -Publisher
FRONTIERS MEDIA SA
DOI: 10.3389/fmolb.2020.578137
Keywords
GNG5; human cortical development; basal progenitor cells; neuronal migration; cerebral organoids
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Funding
- ERA-Net E-Rare (HETEROMICS ERARE) [18-049]
- Max Planck Society
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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 DCHS1 and FAT4, which have been associated with PH, exhibit higher levels of GNG5 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. GNG5, 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 GNG5 recapitulates some of the alterations observed upon downregulation of Dchs1 and Fat4 in mice and human cerebral organoids derived from DCHS1 and FAT4 patients, suggesting a critical role of GNG5 in cortical development.
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