The generation and properties of human cortical organoids as a disease model for malformations of cortical development.
The generation and properties of human cortical organoids as a disease model for malformations of cortical development.
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DOI：
10.4103/1673-5374.369100
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发表时间：
2023-10
影响因子：
6.1
通讯作者：
Miao CY
中科院分区：
文献类型：
--
作者：
Zhang XP;Wang XY;Wang SN;Miao CY
作为三维的“类器官”聚集体，人类皮质类器官已成为研究人类大脑进化和大脑疾病的强大模型，具有人类特异性、保真性和可操作性的独特优势。来源于人类多能干细胞的人类皮质类器官可以在分子、细胞、结构和功能水平上精细地复制人类皮质发育的许多关键特性，包括解剖学、功能神经网络和不同脑区之间的相互作用，从而促进大脑发育和进化的发现。除了研究大脑皮层发育的神经电生理特征外，人类皮层类器官已被广泛用于模拟皮层相关疾病的病理生理特征，特别是在模拟皮层发育畸形方面，从而揭示病理机制和鉴定有效药物。在这篇综述中，我们概述了人类皮质类器官的产生和再现皮质发育的特性，并进一步概述了它们在皮质发育畸形建模中的应用，包括病理表型、潜在机制和救援策略。
As three-dimensional “organ-like” aggregates, human cortical organoids have emerged as powerful models for studying human brain evolution and brain disorders with unique advantages of human-specificity, fidelity and manipulation. Human cortical organoids derived from human pluripotent stem cells can elaborately replicate many of the key properties of human cortical development at the molecular, cellular, structural, and functional levels, including the anatomy, functional neural network, and interaction among different brain regions, thus facilitating the discovery of brain development and evolution. In addition to studying the neuro-electrophysiological features of brain cortex development, human cortical organoids have been widely used to mimic the pathophysiological features of cortical-related disease, especially in mimicking malformations of cortical development, thus revealing pathological mechanism and identifying effective drugs. In this review, we provide an overview of the generation of human cortical organoids and the properties of recapitulated cortical development and further outline their applications in modeling malformations of cortical development including pathological phenotype, underlying mechanisms and rescue strategies.
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影响因子：
16.2
作者：
Geschwind DH;Rakic P
通讯作者：
Rakic P
影响因子：
23.9
作者：
Bershteyn M;Nowakowski TJ;Pollen AA;Di Lullo E;Nene A;Wynshaw-Boris A;Kriegstein AR
通讯作者：
Kriegstein AR
影响因子：
64.5
作者：
Andersen J;Revah O;Miura Y;Thom N;Amin ND;Kelley KW;Singh M;Chen X;Thete MV;Walczak EM;Vogel H;Fan HC;Paşca SP
通讯作者：
Paşca SP
影响因子：
4.3
作者：
Bejoy, Julie;Yuan, Xuegang;Li, Yan
通讯作者：
Li, Yan
DOI：
10.1093/brain/aws019
发表时间：
2012-05
期刊：
Brain : a journal of neurology
影响因子：
--
作者：
Barkovich AJ;Guerrini R;Kuzniecky RI;Jackson GD;Dobyns WB
通讯作者：
Dobyns WB