ArticleEpilepsia2020
Characterization of seizure susceptibility in Pcdh19 mice.
Article in Epilepsia, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed.
- Increased excitability of layer 5 neocortical pyramidal neurons and its contribution to seizure activity in Gabrg2 gene-deficient mice.iScience · 2026Article
- Gut microbiota-bile acid metabolic disorder involved in the cognitive impairments in epilepsy through HO-1 dependent ferroptosis.Journal of pharmaceutical analysis · 2025Article
- Epilepsy Associated Gene,Genes · 2025Article
- Neuronal network activity and connectivity are impaired in a conditional knockout mouse model with PCDH19 mosaic expression.Molecular psychiatry · 2024Article
- Mosaicism-independent mechanisms contribute to Pcdh19-related epilepsy and repetitive behaviors inProceedings of the National Academy of Sciences of the United States of America · 2024Article
- Developmental and epileptic encephalopathies: from genetic heterogeneity to phenotypic continuum.Physiological reviews · 2023Review
- Mosaic and non-mosaic protocadherin 19 mutation leads to neuronal hyperexcitability in zebrafish.Neurobiology of disease · 2022Article
- The epilepsy-associated protein PCDH19 undergoes NMDA receptor-dependent proteolytic cleavage and regulates the expression of immediate-early genes.Cell reports · 2022Article
- Epilepsy surgery in PCDH 19 related developmental and epileptic encephalopathy: A case report.Epilepsy & behavior reports · 2022Article
- A rat model of a focal mosaic expression of PCDH19 replicates human brain developmental abnormalities and behaviours.Brain communications · 2022Article
- Protocadherin 19 Clustering Epilepsy and Neurosteroids: Opportunities for Intervention.International journal of molecular sciences · 2021Review
- Progesterone modulates neuronal excitability bidirectionally.Neuroscience letters · 2021Review
- Inhibition of Death-associated Protein Kinase 1 protects against Epileptic Seizures in mice.International journal of biological sciences · 2021Article
- Review
- Pathogenic variants identified by whole-exome sequencing in 43 patients with epilepsy.Human genomics · 2020Article
- A Case Report of Parental Germline Mosaicism in theBasic and clinical neuroscienceArticle
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4 authors.
Funding
Abstract
objectivePCDH19-related epilepsy is characterized by a distinctive pattern of X-linked inheritance, where heterozygous females exhibit seizures and hemizygous males are asymptomatic. A cellular interference mechanism resulting from the presence of both wild-type and mutant PCDH19 neurons in heterozygous patients or mosaic carriers of PCDH19 variants has been hypothesized. We aim to investigate seizure susceptibility and progression in the Pchd19 mouse model.
methodsWe assessed seizure susceptibility and progression in the Pcdh19 mouse model using three acute seizure induction paradigms. We first induced focal, clonic seizures using the 6-Hz psychomotor test. Mice were stimulated with increasing current intensities and graded according to a modified Racine scale. We next induced generalized seizures using flurothyl or pentylenetetrazol (PTZ), both γ-aminobutyric acid type A receptor function inhibitors, and recorded latencies to myoclonic and generalized tonic-clonic seizures.
resultsPcdh19 knockout and heterozygous females displayed increased seizure susceptibility across all current intensities in the 6-Hz psychomotor test, and increased severity overall. They also exhibited shorter latencies to generalized seizures following flurothyl, but not PTZ, seizure induction. Hemizygous males showed comparable seizure incidence and severity to their wild-type male littermates across all paradigms tested. SIGNIFICANCE: The heightened susceptibility observed in Pcdh19 knockout females suggests additional mechanisms other than cellular interference are at play in PCDH19-related epilepsy. Further experiments are needed to understand the variability in seizure susceptibility so that this model can be best utilized toward development of future therapeutic strategies for PCDH19-related epilepsy.
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