The State Key Laboratory of Medical Molecular Biology, Neuroscience Center, Medical Primates Research Center and Department of Molecular Biology and Biochemistry, Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences, School of Basic Medicine Peking Union Medical College, 100005, Beijing, China.
Institute of Medical Biology, Chinese Academy of Medical Science and Peking Union Medical College, 650118, Kunming, China.
Cell Death Dis. 2019 Mar 25;10(4):287. doi: 10.1038/s41419-019-1517-1.
During cortical development, neuronal migration is one of the most important steps for normal cortical formation and function, and defects in this process cause many brain diseases. However, the molecular mechanisms underlying this process remain largely unknown. In this study, we found that miR-129-5p and miR-129-3p were expressed in both neural progenitor cells and cortical neurons in the developing murine cortex. Moreover, abnormal miR-129 expression could block radial migration of both the deeper layer and upper layer neurons, and impair the multipolar to bipolar transition. However, antagomir-mediated inhibition resulted in overmigration of neurons. In addition, we showed that Fragile X Mental Retardation gene 1 (Fmr1), which is mutated in the autism spectrum disorder fragile X syndrome, is an important regulatory target for miR-129-5p. Furthermore, Fmr1 loss-of-function and gain-of-function experiments showed opposite effects on miR-129 regulation of neuronal migration, and restoring Fmr1 expression could counteract the deleterious effect of miR-129 on neuronal migration. Taken together, our results suggest that miR-129-5p could modulate the expression of fragile X mental retardation 1 protein (FMRP) to ensure normal neuron positioning in the developing cerebral cortex.
在皮质发育过程中,神经元迁移是皮质正常形成和功能的最重要步骤之一,而该过程的缺陷会导致许多脑部疾病。然而,这一过程背后的分子机制在很大程度上仍然未知。在这项研究中,我们发现 miR-129-5p 和 miR-129-3p 均在发育中的鼠皮质的神经祖细胞和皮质神经元中表达。此外,异常的 miR-129 表达可阻断深层和浅层神经元的放射状迁移,并损害多极向双极的转变。然而,反义寡核苷酸抑制导致神经元过度迁移。此外,我们表明,脆性 X 智力低下基因 1(Fmr1)在自闭症谱系障碍脆性 X 综合征中发生突变,是 miR-129-5p 的一个重要调节靶点。此外,Fmr1 功能丧失和功能获得实验显示出对 miR-129 调节神经元迁移的相反作用,恢复 Fmr1 表达可以抵消 miR-129 对神经元迁移的有害影响。总之,我们的结果表明,miR-129-5p 可以调节脆性 X 智力低下蛋白 1(FMRP)的表达,以确保发育中的大脑皮层中正常的神经元定位。