The relationships between impaired cortical development and consequent malformations in neurodevelopmental disorders, as well as the genes implicated in these processes, are not fully elucidated to date. In this study, we report six novel cases of patients affected by BBSOAS (Boonstra‐Bosch‐Schaff optic atrophy syndrome), a newly emerging rare neurodevelopmental disorder, caused by loss‐of‐function mutations of the transcriptional regulator NR2F1. Young patients with NR2F1 haploinsufficiency display mild to moderate intellectual disability and show reproducible polymicrogyria‐like brain malformations in the parietal and occipital cortex. Using a recently established BBSOAS mouse model, we found that Nr2f1 regionally controls long‐term self‐renewal of neural progenitor cells via modulation of cell cycle genes and key cortical development master genes, such as Pax6. In the human fetal cortex, distinct NR2F1 expression levels encompass gyri and sulci and correlate with local degrees of neurogenic activity. In addition, reduced NR2F1 levels in cerebral organoids affect neurogenesis and PAX6 expression. We propose NR2F1 as an area‐specific regulator of mouse and human brain morphology and a novel causative gene of abnormal gyrification.

The detailed study of the low energy secondary electron yield (LE-SEY) of technical Cu for low electron energies (from 0 to 20 eV) is very important for electron cloud build up in high intensity accelerators and in many other fields of research. Different devices base their functionalities on the number of electrons produced by a surface when hit by other electrons, namely its SEY, and, in most cases, on its very low energy behavior. However, LE-SEY has been rarely addressed due to the intrinsic experimental complexity to control very low energy electrons. Furthermore, several results published in the past have been recently questioned, allegedly suffering from experimental systematics. Here, we critically review the experimental method used to study LE-SEY and precisely define the energy region in which the experimental data can be considered valid. By analyzing the significantly different behavior of LE-SEY in clean polycrystalline Cu (going toward zero at zero impinging energies) and in its as received technical counterpart (maintaining a significant value in the entire region), we solve most, if not all, of the apparent controversy present in the literature, producing important inputs for better understanding the device performances related to their LE-SEY. Simulations are then performed to address the impact of such results on electron cloud predictions in the LH

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