Abstract
The primary cilium, a microtubule based organelle protruding from the cell surface and acting as an antenna in multiple signaling pathways, takes center stage in the formation of the cerebral cortex, the part of the brain that performs highly complex neural tasks and confers humans with their unique cognitive capabilities. These activities require dozens of different types of neurons that are interconnected in complex ways. Due to this complexity, corticogenesis has been regarded as one of the most complex developmental processes and cortical malformations underlie a number of neurodevelopmental disorders such as intellectual disability, autism spectrum disorders, and epilepsy. Cortical development involves several steps controlled by cell–cell signaling. In fact, recent findings have implicated cilia in diverse processes such as neurogenesis, neuronal migration, axon pathfinding, and circuit formation in the developing cortex. Here, we will review recent advances on the multiple roles of cilia during cortex formation and will discuss the implications for a better understanding of the disease mechanisms underlying neurodevelopmental disorders.
Highlights
The cerebral cortex contains dozens of different types of neurons
Throughout this review, we have emphasized the importance of ciliary mediated processing of Gli3 for cortical patterning and stem cell development reflecting a strong requirement to suppress Hedgehog signaling in these early steps of corticogenesis
Hedgehog signaling becomes increasingly more active at later stages to control the formation of olfactory bulb interneurons and of glial cells (Zhang et al, 2020) and establishing and maintaining the neurogenic niche in the subventricular zone (SVZ) (Petrova et al, 2013; Wang et al, 2014)
Summary
The cerebral cortex contains dozens of different types of neurons (van den Ameele et al, 2014). The opposite effect, namely a down-regulation of basal progenitor and neuron formation, was observed in mice mutant for Rpgrip1l (Postel et al, 2019), which is crucial for establishing the transition zone (Mahuzier et al, 2012; Reiter et al, 2012; Shi et al, 2017; Wiegering et al, 2018) and for controlling proteasome activity and Gli3 processing at the ciliary base (Gerhardt et al, 2015).
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