Neuronal Defects in a Human Cellular Model of 22q11.2 Deletion Syndrome

22q11.2 deletion syndrome (22q11DS) is a common cause of developmental neuropsychiatric disorders, including psychosis, autism and epilepsy. This highly penetrant genetic syndrome provides a unique opportunity to mitigate the challenges raised by the heterogeneity of complex mental disorders and to identify specific neuronal phenotypes. Here, we generated induced pluripotent stem cells from subjects carrying a 3 Mb deletion at the 22q11.2 locus and from controls and differentiated these cells in vitro into three-dimensional organoid resembling the developing cerebral cortex. We performed single-cell RNA-sequencing to establish the reliability and reproducibility of cortical organoid differentiation in 22q11DS. RNA sequencing of iPS cell-derived cortical spheroids from four human cell lines: two derived from subjects with 22q11.2 Deletion Syndrome and two control subjects. **Submitter declares that raw data were not provided due to confidentiality concerns.**