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Nature ; 621(7978): 373-380, 2023 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-37704762

RESUMEN

The development of the human brain involves unique processes (not observed in many other species) that can contribute to neurodevelopmental disorders1-4. Cerebral organoids enable the study of neurodevelopmental disorders in a human context. We have developed the CRISPR-human organoids-single-cell RNA sequencing (CHOOSE) system, which uses verified pairs of guide RNAs, inducible CRISPR-Cas9-based genetic disruption and single-cell transcriptomics for pooled loss-of-function screening in mosaic organoids. Here we show that perturbation of 36 high-risk autism spectrum disorder genes related to transcriptional regulation uncovers their effects on cell fate determination. We find that dorsal intermediate progenitors, ventral progenitors and upper-layer excitatory neurons are among the most vulnerable cell types. We construct a developmental gene regulatory network of cerebral organoids from single-cell transcriptomes and chromatin modalities and identify autism spectrum disorder-associated and perturbation-enriched regulatory modules. Perturbing members of the BRG1/BRM-associated factor (BAF) chromatin remodelling complex leads to enrichment of ventral telencephalon progenitors. Specifically, mutating the BAF subunit ARID1B affects the fate transition of progenitors to oligodendrocyte and interneuron precursor cells, a phenotype that we confirmed in patient-specific induced pluripotent stem cell-derived organoids. Our study paves the way for high-throughput phenotypic characterization of disease susceptibility genes in organoid models with cell state, molecular pathway and gene regulatory network readouts.


Asunto(s)
Trastorno del Espectro Autista , Encéfalo , Discapacidades del Desarrollo , Organoides , Análisis de Expresión Génica de una Sola Célula , Humanos , Trastorno del Espectro Autista/complicaciones , Trastorno del Espectro Autista/genética , Trastorno del Espectro Autista/patología , Trastorno Autístico/complicaciones , Trastorno Autístico/genética , Trastorno Autístico/patología , Encéfalo/citología , Encéfalo/metabolismo , Linaje de la Célula/genética , Cromatina/genética , Proteína 9 Asociada a CRISPR/metabolismo , Sistemas CRISPR-Cas , Discapacidades del Desarrollo/complicaciones , Discapacidades del Desarrollo/genética , Discapacidades del Desarrollo/patología , Edición Génica , Mutación con Pérdida de Función , Mosaicismo , Neuronas/metabolismo , Neuronas/patología , Organoides/citología , Organoides/metabolismo , ARN Guía de Sistemas CRISPR-Cas , Transcripción Genética
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