ArticleBMC neuroscience2016
Developmental interneuron subtype deficits after targeted loss of Arx.
Article in BMC neuroscience, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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Who cites it
32 citing papers in PubMed, 56 citations in OpenAlex.
- Reduction of forebrain interneurons alters cellular organization and cortical neurogenesis in a mouse model of X-linked intellectual disability.bioRxiv : the preprint server for biology · 2026Article
- Zebrafish models of developmental epileptic encephalopathy accurately reflect clinical electrographic biomarkers.Epilepsia · 2026Article
- Dual developmental effects of ARX poly-alanine mutations on human cortical excitatory and inhibitory neurons.Cell reports · 2026Article
- Single-Molecule Array (Simoa) Technology as a Highly Sensitive Screening Tool for Protein-Protein Interactions.ACS sensors · 2025Article
- Interneuron migration defects during corticogenesis contribute to Dyrk1a haploinsufficiency syndrome pathogenesis.Molecular psychiatry · 2025Article
- Interneuron migration impairment and brain region-specific DNA damage response following irradiation during early neurogenesis in mice.Cellular and molecular life sciences : CMLS · 2025Article
- Arx revisited: involved in the development of GABAergic interneurons.Frontiers in cell and developmental biology · 2025Review
- Cortical interneuron development is affected in 4H leukodystrophy.Brain : a journal of neurology · 2023Article
- Distinct hyperactive RAS/MAPK alleles converge on common GABAergic interneuron core programs.Development (Cambridge, England) · 2023Article
- Cortical Parvalbumin-Positive Interneuron Development and Function Are Altered in the APC Conditional Knockout Mouse Model of Infantile and Epileptic Spasms Syndrome.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2023Article
- Genetic Regulation of Vertebrate Forebrain Development by Homeobox Genes.Frontiers in neuroscience · 2022Review
- Inhibitory synapse dysfunction and epileptic susceptibility associated with KIF2A deletion in cortical interneurons.Frontiers in molecular neuroscience · 2022Article
- Article
- Development, Diversity, and Death of MGE-Derived Cortical Interneurons.International journal of molecular sciences · 2021Review
- The expression of aristaless-related homeobox in neural progenitors of gyrencephalic carnivore ferrets.Biochemistry and biophysics reports · 2021Article
- Phenotypic analysis of catastrophic childhood epilepsy genes.Communications biology · 2021Article
- Article
- Fold Change Detection in Visual Processing.Frontiers in neural circuits · 2021Article
- Heterozygous nonsense ARX mutation in a family highlights the complexity of clinical and molecular diagnosis in case of chromosomal and single gene disorder co-inheritance.Molecular genetics & genomic medicine · 2020Article
- Modeling epileptic spasms during infancy: Are we heading for the treatment yet?Pharmacology & therapeutics · 2020Review
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Authors and funding
7 authors at 2 institutions in 1 country.
Funding
Abstract
backgroundAristaless-related homeobox (ARX) is a paired-like homeodomain transcription factor that functions primarily as a transcriptional repressor and has been implicated in neocortical interneuron specification and migration. Given the role interneurons appear to play in numerous human conditions including those associated with ARX mutations, it is essential to understand the consequences of mutations in this gene on neocortical interneurons. Previous studies have examined the effect of germline loss of Arx, or targeted mutations in Arx, on interneuron development. We now present the effect of conditional loss of Arx on interneuron development.
resultsTo further elucidate the role of Arx in forebrain development we performed a series of anatomical and developmental studies to determine the effect of conditional loss of Arx specifically from developing interneurons in the neocortex and hippocampus. Analysis and cell counts were performed from mouse brains using immunohistochemical and in situ hybridization assays at 4 times points across development. Our data indicate that early in development, instead of a loss of ventral precursors, there is a shift of these precursors to more ventral locations, a deficit that persists in the adult nervous system. The result of this developmental shift is a reduced number of interneurons (all subtypes) at early postnatal and later time periods. In addition, we find that X inactivation is stochastic, and occurs at the level of the neural progenitors.
conclusionThese data provide further support that the role of Arx in interneuron development is to direct appropriate migration of ventral neuronal precursors into the dorsal cortex and that the loss of Arx results in a failure of interneurons to reach the cortex and thus a deficiency in interneurons.
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