ArticleThe Journal of neuroscience : the official journal of the Society for Neuroscience2009
Abnormal network activity in a targeted genetic model of human double cortex.
Article in The Journal of neuroscience : the official journal of the Society for Neuroscience, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers.
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Who cites it
34 citing papers in PubMed, 77 citations in OpenAlex.
- Limitations of genomics to predict and treat autism: a disorder born in the womb.Journal of medical genetics · 2025Review
- Disrupted resting-sate brain network dynamics in children born extremely preterm.Cerebral cortex (New York, N.Y. : 1991) · 2023Article
- An action-concept processing advantage in a patient with a double motor cortex.Brain and cognition · 2022Article
- A deletion in Eml1 leads to bilateral subcortical heterotopia in the tish rat.Neurobiology of disease · 2020Article
- Spontaneous epileptiform activity in a rat model of bilateral subcortical band heterotopia.Epilepsia · 2019Article
- Toward a Better Understanding of Neuronal Migration Deficits in Autism Spectrum Disorders.Frontiers in cell and developmental biology · 2019Review
- Why Malformations of Cortical Development Cause Epilepsy.Frontiers in neuroscience · 2019Article
- Disorders of neurogenesis and cortical development.Dialogues in clinical neuroscience · 2018Review
- Correct Laminar Positioning in the Neocortex Influences Proper Dendritic and Synaptic Development.Cerebral cortex (New York, N.Y. : 1991) · 2018Article
- Pathogenesis of Börjeson-Forssman-Lehmann syndrome: Insights from PHF6 function.Neurobiology of disease · 2016Review
- Neuronal migration disorders: Focus on the cytoskeleton and epilepsy.Neurobiology of disease · 2016Review
- Neuronal Heterotopias Affect the Activities of Distant Brain Areas and Lead to Behavioral Deficits.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2015Article
- Is birth a critical period in the pathogenesis of autism spectrum disorders?Nature reviews. Neuroscience · 2015Review
- Causes and consequences of gray matter heterotopia.CNS neuroscience & therapeutics · 2015Review
- Foxp1 regulates cortical radial migration and neuronal morphogenesis in developing cerebral cortex.PloS one · 2015Article
- MGARP regulates mouse neocortical development via mitochondrial positioning.Molecular neurobiology · 2014Article
- Neurobiology of autism gene products: towards pathogenesis and drug targets.Psychopharmacology · 2014Review
- Dampened hippocampal oscillations and enhanced spindle activity in an asymptomatic model of developmental cortical malformations.Frontiers in systems neuroscience · 2014Article
- Using C. elegans to decipher the cellular and molecular mechanisms underlying neurodevelopmental disorders.Molecular neurobiology · 2013Review
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Corrections and comments
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Authors and funding
8 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
In human patients, cortical dysplasia produced by Doublecortin (DCX) mutations lead to mental retardation and intractable infantile epilepsies, but the underlying mechanisms are not known. DCX(-/-) mice have been generated to investigate this issue. However, they display no neocortical abnormality, lessening their impact on the field. In contrast, in utero knockdown of DCX RNA produces a morphologically relevant cortical band heterotopia in rodents. On this preparation we have now compared the neuronal and network properties of ectopic, overlying, and control neurons in an effort to identify how ectopic neurons generate adverse patterns that will impact cortical activity. We combined dynamic calcium imaging and anatomical and electrophysiological techniques and report now that DCX(-/-)EGFP(+)-labeled ectopic neurons that fail to migrate develop extensive axonal subcortical projections and retain immature properties, and most of them display a delayed maturation of GABA-mediated signaling. Cortical neurons overlying the heterotopia, in contrast, exhibit a massive increase of ongoing glutamatergic synaptic currents reflecting a strong reactive plasticity. Neurons in both experimental fields are more frequently coactive in coherent synchronized oscillations than control cortical neurons. In addition, both fields displayed network-driven oscillations during evoked epileptiform burst. These results show that migration disorders produce major alterations not only in neurons that fail to migrate but also in their programmed target areas. We suggest that this duality play a major role in cortical dysfunction of DCX brains.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.