{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,20]],"date-time":"2026-10-20T03:55:51Z","timestamp":1792468551691,"version":"4.5.0"},"reference-count":30,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2024,11,13]],"date-time":"2024-11-13T00:00:00Z","timestamp":1731456000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100010269","name":"Wellcome Trust","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100010269","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100016063","name":"Institutional Strategic Support Fund","doi-asserted-by":"publisher","award":["BB\/T015705\/1"],"award-info":[{"award-number":["BB\/T015705\/1"]}],"id":[{"id":"10.13039\/100016063","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004359","name":"Swedish Research Council","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004359","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007436","name":"Erling Persson Family Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007436","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100013495","name":"M\u00e4rta and Gunnar V. Philipsons Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100013495","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003748","name":"Svenska S\u00e4llskapet f\u00f6r Medicinsk Forskning","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003748","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,4,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Mutations in the gene encoding the alpha3 Na+\/K+-ATPase isoform (ATP1A3) lead to movement disorders that manifest with dystonia, a common neurological symptom with many different origins, but for which the underlying molecular mechanisms remain poorly understood.<\/jats:p>\n               <jats:p>We have generated an ATP1A3 mutant mouse that displays motor impairments and a hyperexcitable motor phenotype compatible with dystonia. We show that neurons harbouring this mutation are compromised in their ability to extrude raised levels of intracellular sodium, highlighting a profound deficit in neuronal sodium homeostasis. We show that the spinal motor network in ATP1A3 mutant mice has a reduced responsiveness to activity-dependent rises in intracellular sodium and that this is accompanied by loss of the Na+\/K+-ATPase-mediated afterhyperpolarization in motor neurons.<\/jats:p>\n               <jats:p>Taken together, our data support that the alpha3 Na+\/K+-ATPase is important for cellular and spinal motor network homeostasis. These insights suggest that it may be useful to consider ways to compensate for this loss of a critical afterhyperpolarization-dependent control of neuronal excitability when developing future therapies for dystonia.<\/jats:p>","DOI":"10.1093\/brain\/awae373","type":"journal-article","created":{"date-parts":[[2024,11,13]],"date-time":"2024-11-13T06:02:41Z","timestamp":1731477761000},"page":"1099-1105","source":"Crossref","is-referenced-by-count":8,"title":["ATP1A3 dysfunction causes motor hyperexcitability and afterhyperpolarization loss in a dystonia model"],"prefix":"10.1093","volume":"148","author":[{"given":"Evgeny E","family":"Akkuratov","sequence":"first","affiliation":[{"name":"Science for Life Laboratory, Department of Women\u2019s and Children\u2019s Health, Karolinska Institutet , Solna 171 21 ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Sorrell","sequence":"additional","affiliation":[{"name":"School of Psychology 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Technology , Solna 171 21 ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lill-Britt","family":"Svensson","sequence":"additional","affiliation":[{"name":"Science for Life Laboratory, Department of Women\u2019s and Children\u2019s Health, Karolinska Institutet , Solna 171 21 ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Lindskog","sequence":"additional","affiliation":[{"name":"Department of Medical Cell Biology, Uppsala University , Uppsala 751 23 ,","place":["Sweden"]},{"name":"Department of Neurobiology, Care Sciences and Society, Karolinska Institutet , Solna 171 21 ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"Fritz","sequence":"additional","affiliation":[{"name":"Science for Life Laboratory, Department of Women\u2019s and Children\u2019s Health, Karolinska Institutet , Solna 171 21 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Medicine, Department for Child Neurology, Developmental Neurology, General Pediatrics, Endocrinology, Diabetology, Social Pediatrics, University Hospital and Faculty of Medicine, Eberhard Karls University T\u00fcbingen , T\u00fcbingen 72076 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6727-3802","authenticated-orcid":false,"given":"Per","family":"Svenningsson","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Karolinska Institutet , Solna 171 77 ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hjalmar","family":"Brismar","sequence":"additional","affiliation":[{"name":"Science for Life Laboratory, Department of Women\u2019s and Children\u2019s Health, Karolinska Institutet , Solna 171 21 ,","place":["Sweden"]},{"name":"Science for Life Laboratory, Department of Applied Physics, KTH Royal Institute of Technology , Solna 171 21 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