Program in Neurodevelopment and Regeneration, Yale University School of Medicine, New Haven, CT 06520, USA.
Proc Natl Acad Sci U S A. 2013 Jul 23;110(30):12361-6. doi: 10.1073/pnas.1309725110. Epub 2013 Jul 8.
Human embryonic stem cells (hESCs) can be induced and differentiated to form a relatively homogeneous population of neuronal precursors in vitro. We have used this system to screen for genes necessary for neural lineage development by using a pooled human short hairpin RNA (shRNA) library screen and massively parallel sequencing. We confirmed known genes and identified several unpredicted genes with interrelated functions that were specifically required for the formation or survival of neuronal progenitor cells without interfering with the self-renewal capacity of undifferentiated hESCs. Among these are several genes that have been implicated in various neurodevelopmental disorders (i.e., brain malformations, mental retardation, and autism). Unexpectedly, a set of genes mutated in late-onset neurodegenerative disorders and with roles in the formation of RNA granules were also found to interfere with neuronal progenitor cell formation, suggesting their functional relevance in early neurogenesis. This study advances the feasibility and utility of using pooled shRNA libraries in combination with next-generation sequencing for a high-throughput, unbiased functional genomic screen. Our approach can also be used with patient-specific human-induced pluripotent stem cell-derived neural models to obtain unparalleled insights into developmental and degenerative processes in neurological or neuropsychiatric disorders with monogenic or complex inheritance.
人胚胎干细胞(hESCs)可以在体外诱导分化为相对均一的神经前体细胞群体。我们使用该系统通过高通量的 pooled human short hairpin RNA(shRNA)文库筛选和大规模平行测序来筛选神经谱系发育所必需的基因。我们验证了已知基因,并鉴定了几个具有相互关联功能的未预测基因,这些基因对于神经祖细胞的形成或存活是必需的,而不干扰未分化的 hESC 的自我更新能力。其中一些基因与各种神经发育障碍(例如脑畸形、智力迟钝和自闭症)有关。出乎意料的是,一组在迟发性神经退行性疾病中突变的、在 RNA 颗粒形成中起作用的基因也被发现干扰神经祖细胞的形成,这表明它们在早期神经发生中的功能相关性。这项研究推进了使用 pooled shRNA 文库结合下一代测序进行高通量、无偏功能基因组筛选的可行性和实用性。我们的方法也可以用于患者特异性的人诱导多能干细胞衍生的神经模型,以获得对具有单基因或复杂遗传的神经或神经精神疾病的发育和退行过程的独特见解。