Avila A, Vidal P M, Tielens S, Morelli G, Laguesse S, Harvey R J, Rigo J-M, Nguyen L
1] BIOMED Research Institute, Hasselt University, Diepenbeek, Belgium [2] GIGA-Neurosciences, University of Liège, C.H.U. Sart Tilman, Liège, Belgium [3] Interdisciplinary Cluster for Applied Genoproteomics (GIGA-R), University of Liège, C.H.U. Sart Tilman, Liège, Belgium.
BIOMED Research Institute, Hasselt University, Diepenbeek, Belgium.
Cell Death Differ. 2014 Nov;21(11):1696-708. doi: 10.1038/cdd.2014.75. Epub 2014 Jun 13.
The development of the cerebral cortex requires coordinated regulation of proliferation, specification, migration and differentiation of cortical progenitors into functionally integrated neurons. The completion of the neurogenic program requires a dynamic interplay between cell intrinsic regulators and extrinsic cues, such as growth factor and neurotransmitters. We previously demonstrated a role for extrasynaptic glycine receptors (GlyRs) containing the α2 subunit in cerebral cortical neurogenesis, revealing that endogenous GlyR activation promotes interneuron migration in the developing cortical wall. The proliferative compartment of the cortex comprises apical progenitors that give birth to neurons directly or indirectly through the generation of basal progenitors, which serve as amplification step to generate the bulk of cortical neurons. The present work shows that genetic inactivation of Glra2, the gene coding the α2 subunit of GlyRs, disrupts dorsal cortical progenitor homeostasis with an impaired capability of apical progenitors to generate basal progenitors. This defect results in an overall reduction of projection neurons that settle in upper or deep layers of the cerebral cortex. Overall, the depletion of cortical neurons observed in Glra2-knockout embryos leads to moderate microcephaly in newborn Glra2-knockout mice. Taken together, our findings support a contribution of GlyR α2 to early processes in cerebral cortical neurogenesis that are required later for the proper development of cortical circuits.
大脑皮层的发育需要对皮质祖细胞的增殖、特化、迁移和分化进行协调调控,使其分化为功能整合的神经元。神经发生程序的完成需要细胞内在调节因子与外在信号(如生长因子和神经递质)之间的动态相互作用。我们之前证明了含有α2亚基的突触外甘氨酸受体(GlyRs)在大脑皮质神经发生中发挥作用,揭示了内源性GlyR激活促进发育中皮质壁内中间神经元的迁移。皮质的增殖区室包括顶端祖细胞,它们直接或通过产生基底祖细胞间接产生神经元,基底祖细胞作为扩增步骤来产生大部分皮质神经元。目前的研究表明,编码GlyRsα2亚基的基因Glra2的基因失活会破坏背侧皮质祖细胞的稳态,顶端祖细胞产生基底祖细胞的能力受损。这一缺陷导致定居在大脑皮层上层或深层的投射神经元总体减少。总体而言,在Glra2基因敲除胚胎中观察到的皮质神经元减少导致新生Glra2基因敲除小鼠出现中度小头畸形。综上所述,我们的研究结果支持GlyRα2在大脑皮质神经发生早期过程中的作用,而这些过程对于皮质回路的正常发育是必需的。