@article{
 title = {Developmentally regulated impairment of parvalbumin interneuron synaptic transmission in an experimental model of Dravet syndrome},
 type = {article},
 year = {2022},
 keywords = {Dravet syndrome,GABAergic interneurons,Nav1.1,SCN1A,epilepsy},
 pages = {110580},
 volume = {38},
 websites = {https://linkinghub.elsevier.com/retrieve/pii/S2211124722003242},
 month = {3},
 publisher = {Cell Press},
 day = {29},
 id = {833062c0-d840-3c91-bd45-c19420d31d66},
 created = {2022-03-30T11:50:34.278Z},
 accessed = {2022-03-30},
 file_attached = {true},
 profile_id = {14badb0b-89a9-3047-a69a-683d5240354c},
 last_modified = {2022-05-12T14:12:26.991Z},
 read = {true},
 starred = {false},
 authored = {true},
 confirmed = {true},
 hidden = {false},
 folder_uuids = {11b3f8d2-36c2-431a-ba82-dee5f10d76b7},
 private_publication = {false},
 abstract = {Dravet syndrome is a neurodevelopmental disorder characterized by epilepsy, intellectual disability, and sudden death due to pathogenic variants in SCN1A with loss of function of the sodium channel subunit Nav1.1. Nav1.1-expressing parvalbumin GABAergic interneurons (PV-INs) from young Scn1a+/− mice show impaired action potential generation. An approach assessing PV-IN function in the same mice at two time points shows impaired spike generation in all Scn1a+/− mice at postnatal days (P) 16–21, whether deceased prior or surviving to P35, with normalization by P35 in surviving mice. However, PV-IN synaptic transmission is dysfunctional in young Scn1a+/− mice that did not survive and in Scn1a+/− mice ≥ P35. Modeling confirms that PV-IN axonal propagation is more sensitive to decreased sodium conductance than spike generation. These results demonstrate dynamic dysfunction in Dravet syndrome: combined abnormalities of PV-IN spike generation and propagation drives early disease severity, while ongoing dysfunction of synaptic transmission contributes to chronic pathology.},
 bibtype = {article},
 author = {Kaneko, Keisuke and Currin, Christopher B. and Goff, Kevin M. and Wengert, Eric R. and Somarowthu, Ala and Vogels, Tim P. and Goldberg, Ethan M.},
 doi = {10.1016/j.celrep.2022.110580},
 journal = {Cell Reports},
 number = {13}
}

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Nav1.1-expressing parvalbumin GABAergic interneurons (PV-INs) from young Scn1a+/− mice show impaired action potential generation. An approach assessing PV-IN function in the same mice at two time points shows impaired spike generation in all Scn1a+/− mice at postnatal days (P) 16–21, whether deceased prior or surviving to P35, with normalization by P35 in surviving mice. However, PV-IN synaptic transmission is dysfunctional in young Scn1a+/− mice that did not survive and in Scn1a+/− mice ≥ P35. Modeling confirms that PV-IN axonal propagation is more sensitive to decreased sodium conductance than spike generation. These results demonstrate dynamic dysfunction in Dravet syndrome: combined abnormalities of PV-IN spike generation and propagation drives early disease severity, while ongoing dysfunction of synaptic transmission contributes to chronic pathology.","bibtype":"article","author":"Kaneko, Keisuke and Currin, Christopher B. and Goff, Kevin M. and Wengert, Eric R. and Somarowthu, Ala and Vogels, Tim P. and Goldberg, Ethan M.","doi":"10.1016/j.celrep.2022.110580","journal":"Cell Reports","number":"13","bibtex":"@article{\n title = {Developmentally regulated impairment of parvalbumin interneuron synaptic transmission in an experimental model of Dravet syndrome},\n type = {article},\n year = {2022},\n keywords = {Dravet syndrome,GABAergic interneurons,Nav1.1,SCN1A,epilepsy},\n pages = {110580},\n volume = {38},\n websites = {https://linkinghub.elsevier.com/retrieve/pii/S2211124722003242},\n month = {3},\n publisher = {Cell Press},\n day = {29},\n id = {833062c0-d840-3c91-bd45-c19420d31d66},\n created = {2022-03-30T11:50:34.278Z},\n accessed = {2022-03-30},\n file_attached = {true},\n profile_id = {14badb0b-89a9-3047-a69a-683d5240354c},\n last_modified = {2022-05-12T14:12:26.991Z},\n read = {true},\n starred = {false},\n authored = {true},\n confirmed = {true},\n hidden = {false},\n folder_uuids = {11b3f8d2-36c2-431a-ba82-dee5f10d76b7},\n private_publication = {false},\n abstract = {Dravet syndrome is a neurodevelopmental disorder characterized by epilepsy, intellectual disability, and sudden death due to pathogenic variants in SCN1A with loss of function of the sodium channel subunit Nav1.1. 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