{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,15]],"date-time":"2026-04-15T10:10:34Z","timestamp":1776247834077,"version":"3.50.1"},"reference-count":62,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2026,4,15]],"date-time":"2026-04-15T00:00:00Z","timestamp":1776211200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100007857","name":"IDDRC","doi-asserted-by":"publisher","award":["P50 HD103538"],"award-info":[{"award-number":["P50 HD103538"]}],"id":[{"id":"10.13039\/100007857","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Faneca 66 Foundation"},{"name":"Celebrate Hope Foundation"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Sturge\u2013Weber Syndrome (SWS) is a rare neurovascular disorder caused by a somatic mosaic missense point mutation in GNAQ, resulting in a facial capillary malformation (port-wine birthmark) and abnormal blood vessels in the eye and brain. Symptoms include seizures, stroke-like episodes, and glaucoma. Acute seizures induced with low-dose kainate in a transgenic GNAQ R183Q mouse model of SWS assessed seizure susceptibility and the impact of seizures on cerebral vasculature. Mice expressing human GNAQ mutation experienced more severe seizures and greater seizure-induced mortality compared to littermate controls. Mutant mice had longer cortical microvessels, with larger diameters; the expression of tight junction proteins was reduced 2 days after seizures. Blood\u2013brain barrier permeability was not different from controls after chronic gene expression, although vascular dilation persisted compared to controls. These data demonstrate increased seizure susceptibility in this somatic mosaic model of SWS, bidirectional interactions between seizure and vascular remodeling, and chronic persistence of vascular malformation.<\/jats:p>","DOI":"10.3390\/ijms27083519","type":"journal-article","created":{"date-parts":[[2026,4,15]],"date-time":"2026-04-15T09:15:59Z","timestamp":1776244559000},"page":"3519","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Acute Seizure Susceptibility and Chronic Vascular Malformation in a Developmental Mouse Model of Sturge\u2013Weber Syndrome"],"prefix":"10.3390","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0009-0005-0330-7512","authenticated-orcid":false,"given":"Nicholas","family":"Truver","sequence":"first","affiliation":[{"name":"Department of Neurology and Developmental Medicine, Hugo Moser Kennedy Krieger Research Institute, Baltimore, MD 21205, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chase","family":"Solomon","sequence":"additional","affiliation":[{"name":"Department of Neurology and Developmental Medicine, Hugo Moser Kennedy Krieger Research Institute, Baltimore, MD 21205, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anne","family":"Comi","sequence":"additional","affiliation":[{"name":"Department of Neurology and Developmental Medicine, Hugo Moser Kennedy Krieger Research Institute, Baltimore, MD 21205, USA"},{"name":"Department of Neurology, Johns Hopkins School of Medicine, Baltimore, MD 21205, USA"},{"name":"Department of Pediatrics, Johns Hopkins School of Medicine, Baltimore, MD 21205, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2026,4,15]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"3431","DOI":"10.1093\/brain\/awab470","article-title":"Characteristics, Surgical Outcomes, and Influential Factors of Epilepsy in Sturge-Weber Syndrome","volume":"145","author":"Wang","year":"2022","journal-title":"Brain J. 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