Recapitulating cortical development with organoid culture in vitro and modeling abnormal spindle-like (ASPM related primary) microcephaly disease.
Recapitulating cortical development with organoid culture in vitro and modeling abnormal spindle-like (ASPM related primary) microcephaly disease.
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通过体外类器官培养再现皮质发育并模拟异常纺锤样（ASPM 相关原发性）小头畸形疾病
DOI：
10.1007/s13238-017-0479-2
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发表时间：
2017-11
期刊：
影响因子：
21.1
通讯作者：
Wang X
中科院分区：
文献类型：
--
作者：
Li R;Sun L;Fang A;Li P;Wu Q;Wang X
脑类器官体外培养的发展为产生人脑样器官提供了一个机会，以研究人类疾病的机制，这些机制与正在发育的新皮层的心室区（VZ）和室下区（SVZ）的放射状胶质（RG）和外放射状胶质（oRG）细胞的神经发生有关。在早期发育阶段对神经元祖细胞和产生成熟皮层下神经元亚型的组织进行建模对于研究人类大脑发育性疾病至关重要。之前的一些研究已经成功地证明了在培养皿中培养神经类器官，然而，我们展示了一个新的范式，概括了新皮层的发育过程，包括VZ、OSVZ的形成和皮层层结构的层压组织。此外，我们利用来自原发性小头畸形患者的具有Aspm基因功能障碍的患者特异性诱导多能干细胞（iPSCs），证明神经发生缺陷导致患者类器官中神经元活性缺陷，为研究人类中枢神经系统发育疾病提供了一种新的策略。本文的在线版本（doi:10.1007/s13238-017-0479-2）包含补充材料，仅供授权用户使用。
The development of a cerebral organoid culture in vitro offers an opportunity to generate human brain-like organs to investigate mechanisms of human disease that are specific to the neurogenesis of radial glial (RG) and outer radial glial (oRG) cells in the ventricular zone (VZ) and subventricular zone (SVZ) of the developing neocortex. Modeling neuronal progenitors and the organization that produces mature subcortical neuron subtypes during early stages of development is essential for studying human brain developmental diseases. Several previous efforts have shown to grow neural organoid in culture dishes successfully, however we demonstrate a new paradigm that recapitulates neocortical development process with VZ, OSVZ formation and the lamination organization of cortical layer structure. In addition, using patient-specific induced pluripotent stem cells (iPSCs) with dysfunction of the Aspm gene from a primary microcephaly patient, we demonstrate neurogenesis defects result in defective neuronal activity in patient organoids, suggesting a new strategy to study human developmental diseases in central nerve system. The online version of this article (doi:10.1007/s13238-017-0479-2) contains supplementary material, which is available to authorized users.
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