Department of Basic Neurosciences, University of Geneva Medical School, CH-1211, Geneva 4, Switzerland.
Nat Commun. 2017 Oct 27;8(1):1158. doi: 10.1038/s41467-017-01046-w.
Perturbed neuronal migration and circuit development have been implicated in the pathogenesis of neurodevelopmental diseases; however, the direct steps linking these developmental errors to behavior alterations remain unknown. Here we demonstrate that Wnt/C-Kit signaling is a key regulator of glia-guided radial migration in rat somatosensory cortex. Transient downregulation of Wnt signaling in migrating, callosal projection neurons results in delayed positioning in layer 2/3. Delayed neurons display reduced neuronal activity with impaired afferent connectivity causing permanent deficit in callosal projections. Animals with these defects exhibit altered somatosensory function with reduced social interactions and repetitive movements. Restoring normal migration by overexpressing the Wnt-downstream effector C-Kit or selective chemogenetic activation of callosal projection neurons during a critical postnatal period prevents abnormal interhemispheric connections as well as behavioral alterations. Our findings identify a link between defective canonical Wnt signaling, delayed neuronal migration, deficient interhemispheric connectivity and abnormal social behavior analogous to autistic characteristics in humans.
神经迁移和回路发育障碍与神经发育性疾病的发病机制有关;然而,将这些发育错误与行为改变直接联系起来的步骤尚不清楚。在这里,我们证明 Wnt/C-Kit 信号是调节大鼠体感皮层神经胶质导向放射状迁移的关键调控因子。短暂下调迁移中的胼胝体投射神经元中的 Wnt 信号会导致其在第 2/3 层中的定位延迟。延迟的神经元表现出活动减少,传入连接受损,导致胼胝体投射永久性缺失。具有这些缺陷的动物表现出体感功能改变,社交互动减少和重复运动增加。通过过表达 Wnt 下游效应物 C-Kit 或在关键的出生后时期选择性地化学遗传学激活胼胝体投射神经元来恢复正常的迁移,可以防止异常的半球间连接以及行为改变。我们的研究结果表明,在人类中类似于自闭症特征的缺陷性经典 Wnt 信号、神经元迁移延迟、半球间连接缺陷和异常社会行为之间存在联系。