Developmental Neurobiology, Max Planck Institute of Psychiatry, 80804 Munich, Germany.
Graduate School of Systemic Neurosciences, Ludwig-Maximilians-University Munich, 82152 Planegg, Germany.
Development. 2019 Jan 9;146(1):dev163766. doi: 10.1242/dev.163766.
Neuronal migration is a fundamental process that governs embryonic brain development. As such, mutations that affect essential neuronal migration processes lead to severe brain malformations, which can cause complex and heterogeneous developmental and neuronal migration disorders. Our fragmented knowledge about the aetiology of these disorders raises numerous issues. However, many of these can now be addressed through studies of and models that attempt to recapitulate human-specific mechanisms of cortical development. In this Review, we discuss the advantages and limitations of these model systems and suggest that a complementary approach, using combinations of and models, will broaden our knowledge of the molecular and cellular mechanisms that underlie defective neuronal positioning in the human cerebral cortex.
神经元迁移是一个基本过程,它控制着胚胎大脑的发育。因此,影响基本神经元迁移过程的突变会导致严重的脑畸形,从而导致复杂和异质的发育和神经元迁移障碍。我们对这些疾病病因的零碎认识提出了许多问题。然而,通过研究和试图重现皮质发育的人类特异性机制的模型,现在可以解决其中的许多问题。在这篇综述中,我们讨论了这些模型系统的优缺点,并提出了一种补充方法,即使用和模型的组合,这将拓宽我们对导致人类大脑皮层中神经元定位缺陷的分子和细胞机制的认识。