ARX regulates interneuron subtype differentiation and migration
Cho, G.; Lim, Y.; Akula, S. K.; Myers, A. K.; Chen, C.; Rafael, K.; Walsh, C. A.; Golden, J. A.
Show abstract
Mutations in aristaless-related homeobox (ARX) are associated with neurodevelopmental disorders including developmental epilepsies, intellectual disabilities, and autism spectrum disorders, with or without brain malformations. Aspects of these disorders have been linked to abnormal cortical interneuron (cIN) development and function. To further understand ARXs role in cIN development, multiple Arx mutant mouse lines were interrogated. We found that ARX is critical for controlling cIN numbers and distribution, especially, in the developing marginal zone (MZ). Single cell transcriptomics and ChIP-seq, combined with functional studies, revealed ARX directly or indirectly regulates genes involved in proliferation and the cell cycle (e.g., Bub3, Cspr3), fate specification (e.g., Nkx2.1, Maf, Mef2c), and migration (e.g., Nkx2.1, Lmo1, Cxcr4, Nrg1, ErbB4). Our data suggest that the MZ stream defects primarily result from disordered cell-cell communication. Together our findings provide new insights into the mechanisms underlying cIN development and migration and how they are disrupted in several disorders.
Matching journals
The top 6 journals account for 50% of the predicted probability mass.
Similar papers in this journal
Similar papers in this journal
- The patient-specific mouse model with Foxg1 frameshift mutation provides insights into the pathophysiology of FOXG1 syndrome 96%
- Quantitative Cellular-Resolution Map of the Oxytocin Receptor in Postnatally Developing Mouse Brains 96%
- Age-dependent regulation of axoglial interactions and behavior by oligodendrocyte AnkyrinG 96%
Similar papers in this journal
- Enhanced FGFR3 activity in post-mitotic principal neurons during brain development results in cortical dysplasia and axon miswiring 97%
- Early loss of Scribble affects cortical development and interhemispheric connectivity resulting in psychomotor dysregulation. 96%
- Physiological significance of proteolytic processing of Reelin revealed by cleavage-resistant Reelin knock-in mice 96%
Similar papers in this journal
- YTHDF2 in dentate gyrus is the m6A reader mediating m6A modification in hippocampus-dependent learning and memory 95%
- Autism-linked Cullin3 germline haploinsufficiency impacts cytoskeletal dynamics and cortical neurogenesis through RhoA signaling 95%
- Analyses of the Autism-associated Neuroligin-3 R451C Mutation in Human Neurons Reveals a Gain-of-Function Synaptic Mechanism 95%
Similar papers in this journal
- Sustained Generation of Neurons Destined for Neocortex with Oxidative Metabolic Upregulation upon Filamin Abrogation 96%
- Impaired KDM2B-mediated PRC1 recruitment to chromatin causes neural stem cell senescence and ASD/ID-like behavioral deficits 95%
- Cerebellar white matter development is regulated by fractalkine-dependent microglia phagocytosis of oligodendrocyte progenitor cells 94%
"Similar papers" are the closest papers from that journal in the model's embedding space. They show what the match is built on, but the ranking comes mostly from a classifier over the whole training set, not from these examples alone.