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Abstract：
In vitro differentiation of human pluripotent stem cells (hPSCs) into specialized tissues and organs represents a powerful approach to gain insight into those cellular and molecular mechanisms regulating human development. Although normal embryonic eye development is a complex process, generation of ocular organoids and specific ocular tissues from pluripotent stem cells has provided invaluable insights into the formation of lineage-committed progenitor cell populations, signal transduction pathways, and self-organization principles. This review provides a comprehensive summary of recent advances in generation of adenohypophyseal, olfactory, and lens placodes, lens progenitor cells and three-dimensional (3D) primitive lenses, \"lentoid bodies\", and \"micro-lenses\". These cells are produced alone or \"community-grown\" with other ocular tissues. Lentoid bodies/micro-lenses generated from human patients carrying mutations in crystallin genes demonstrate proof-of-principle that these cells are suitable for mechanistic studies of cataractogenesis. Taken together, current and emerging advanced in vitro differentiation methods pave the road to understand molecular mechanisms of cataract formation caused by the entire spectrum of mutations in DNA-binding regulatory genes, such as PAX6, SOX2, FOXE3, MAF, PITX3, and HSF4, individual crystallins, and other genes such as BFSP1, BFSP2, EPHA2, GJA3, GJA8, LIM2, MIP, and TDRD7 represented in human cataract patients.
摘要：
人多能干细胞(hPSC)体外分化为专门的组织和器官代表了一种有效的方法，可以深入了解调节人类发育的细胞和分子机制。虽然正常的胚胎眼发育是一个复杂的过程，从多能干细胞产生眼部类器官和特定的眼部组织提供了宝贵的见解，形成谱系定向祖细胞群，信号转导途径，和自组织原则。这篇综述提供了腺垂体生成的最新进展的全面总结，嗅觉,和镜头拉的话，晶状体祖细胞和三维(3D)原始晶状体，\"扁桃体\",和“微透镜”。这些细胞单独产生或与其他眼组织“社区生长”。从携带晶状体蛋白基因突变的人类患者产生的类牙体/微透镜证明了这些细胞适用于白内障发生的机理研究的原理证明。一起来看,当前和新兴的先进的体外分化方法为理解由DNA结合调节基因的整个突变谱引起的白内障形成的分子机制铺平了道路。如PAX6，SOX2，FOXE3，MAF，PITX3和HSF4，单个晶体蛋白，和其他基因如BFSP1，BFSP2，EPHA2，GJA3，GJA8，LIM2，MIP，和TDRD7在人类白内障患者中代表。
