{"entity": "researcher", "timestamp": "2026-04-16T16:52:28.105Z", "family": "Fritz", "given": "Nicolas", "initials": "N", "orcid": "0000-0002-9722-7425", "affiliations": ["Science for Life Laboratory, Department of Applied Physics, Royal Institute of Technology, 17121 Solna, Sweden. nicolas.fritz@scilifelab.se."], "links": {"self": {"href": "https://publications.scilifelab.se/researcher/ec61383c813443f29dea569ea935b284.json"}, "display": {"href": "https://publications.scilifelab.se/researcher/ec61383c813443f29dea569ea935b284"}}, "publications": [{"entity": "publication", "iuid": "859055d5fe394252bb2808f0d2a3394e", "links": {"self": {"href": "https://publications.scilifelab.se/publication/859055d5fe394252bb2808f0d2a3394e.json"}, "display": {"href": "https://publications.scilifelab.se/publication/859055d5fe394252bb2808f0d2a3394e"}}, "title": "ATP1A3 dysfunction causes motor hyperexcitability and afterhyperpolarization loss in a dystonia model.", "authors": [{"family": "Akkuratov", "given": "Evgeny E", "initials": "EE"}, {"family": "Sorrell", "given": "Francesca", "initials": "F"}, {"family": "Picton", "given": "Laurence D", "initials": "LD"}, {"family": "Sousa", "given": "Vasco C", "initials": "VC"}, {"family": "Paucar", "given": "Martin", "initials": "M", "orcid": "0000-0003-3735-1480", "researcher": {"href": "https://publications.scilifelab.se/researcher/bbc592904eb5402ea48a624471d4b939.json"}}, {"family": "Jans", "given": "Daniel", "initials": "D", "orcid": "0000-0002-6356-9742", "researcher": {"href": "https://publications.scilifelab.se/researcher/fe197a6a51a946a7a6c01f4c9c6cce08.json"}}, {"family": "Svensson", "given": "Lill-Britt", "initials": "LB"}, {"family": "Lindskog", "given": "Maria", "initials": "M", "orcid": "0000-0001-9484-1983", "researcher": {"href": "https://publications.scilifelab.se/researcher/1592b4e2f1354bdd8b35f384dcac78c2.json"}}, {"family": "Fritz", "given": "Nicolas", "initials": "N", "orcid": "0000-0002-9722-7425", "researcher": {"href": "https://publications.scilifelab.se/researcher/ec61383c813443f29dea569ea935b284.json"}}, {"family": "Liebmann", "given": "Thomas", "initials": "T"}, {"family": "Sillar", "given": "Keith T", "initials": "KT"}, {"family": "Rosewich", "given": "Hendrik", "initials": "H", "orcid": "0000-0003-4692-5511", "researcher": {"href": "https://publications.scilifelab.se/researcher/fffad5ec5d2d48a78573e71d7ba3c2dd.json"}}, {"family": "Svenningsson", "given": "Per", "initials": "P", "orcid": "0000-0001-6727-3802", "researcher": {"href": "https://publications.scilifelab.se/researcher/5199496295334771ba3c5621a83a6f43.json"}}, {"family": "Brismar", "given": "Hjalmar", "initials": "H", "orcid": "0000-0003-0578-4003", "researcher": {"href": "https://publications.scilifelab.se/researcher/1ec23336e2ef4e298f340876f1136dce.json"}}, {"family": "Miles", "given": "Gareth B", "initials": "GB"}, {"family": "Aperia", "given": "Anita", "initials": "A"}], "type": "journal article", "published": "2025-04-03", "journal": {"title": "Brain", "issn": "1460-2156", "volume": "148", "issue": "4", "pages": "1099-1105", "issn-l": "0006-8950"}, "abstract": "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. 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. 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.", "doi": "10.1093/brain/awae373", "pmid": "39533828", "labels": {"Integrated Microscopy Technologies Stockholm": "Service"}, "xrefs": [{"db": "pmc", "key": "PMC11967811"}, {"db": "pii", "key": "7896743"}], "notes": [], "created": "2024-11-18T09:02:43.236Z", "modified": "2025-11-12T11:33:48.972Z"}, {"entity": "publication", "iuid": "14baba8c484647cab8b7b0c3ca496a74", "links": {"self": {"href": "https://publications.scilifelab.se/publication/14baba8c484647cab8b7b0c3ca496a74.json"}, "display": {"href": "https://publications.scilifelab.se/publication/14baba8c484647cab8b7b0c3ca496a74"}}, "title": "Regulation of Neuronal Na,K-ATPase by Extracellular Scaffolding Proteins.", "authors": [{"family": "Liebmann", "given": "Thomas", "initials": "T"}, {"family": "Fritz", "given": "Nicolas", "initials": "N", "orcid": "0000-0002-9722-7425", "researcher": {"href": "https://publications.scilifelab.se/researcher/ec61383c813443f29dea569ea935b284.json"}}, {"family": "Kruusm\u00e4gi", "given": "Markus", "initials": "M"}, {"family": "Westin", "given": "Linda", "initials": "L"}, {"family": "Bernhem", "given": "Kristoffer", "initials": "K"}, {"family": "Bondar", "given": "Alexander", "initials": "A", "orcid": "0000-0001-9181-0487", "researcher": {"href": "https://publications.scilifelab.se/researcher/28a9004c16cc4e8fad57a07a986be0b8.json"}}, {"family": "Aperia", "given": "Anita", "initials": "A"}, {"family": "Brismar", "given": "Hjalmar", "initials": "H", "orcid": "0000-0003-0578-4003", "researcher": {"href": "https://publications.scilifelab.se/researcher/1ec23336e2ef4e298f340876f1136dce.json"}}], "type": "journal article", "published": "2018-07-29", "journal": {"volume": "19", "issn": "1422-0067", "issue": "8", "pages": "2214", "title": "Int J Mol Sci", "issn-l": null}, "abstract": "Neuronal activity leads to an influx of Na\u207a that needs to be rapidly cleared. The sodium-potassium ATPase (Na,K-ATPase) exports three Na\u207a ions and imports two K\u207a ions at the expense of one ATP molecule. Na,K-ATPase turnover accounts for the majority of energy used by the brain. To prevent an energy crisis, the energy expense for Na\u207a clearance must provide an optimal effect. Here we report that in rat primary hippocampal neurons, the clearance of Na\u207a ions is more efficient if Na,K-ATPase is laterally mobile in the membrane than if it is clustered. Using fluorescence recovery after photobleaching and single particle tracking analysis, we show that the ubiquitous \u03b11 and the neuron-specific \u03b13 catalytic subunits as well as the supportive \u03b21 subunit of Na,K-ATPase are highly mobile in the plasma membrane. We show that cross-linking of the \u03b21 subunit with polyclonal antibodies or exposure to Modulator of Na,K-ATPase (MONaKA), a secreted protein which binds to the extracellular domain of the \u03b2 subunit, clusters the \u03b13 subunit in the membrane and restricts its mobility. We demonstrate that clustering, caused by cross-linking or by exposure to MONaKA, reduces the efficiency in restoring intracellular Na\u207a. These results demonstrate that extracellular interactions with Na,K-ATPase regulate the Na\u207a extrusion efficiency with consequences for neuronal energy balance.", "doi": "10.3390/ijms19082214", "pmid": "30060621", "labels": {"Integrated Microscopy Technologies Stockholm": "Service", "Bioinformatics Support for Computational Resources": "Service", "Protein Science Facility (PSF)": "Service"}, "xrefs": [{"db": "pmc", "key": "PMC6121408"}, {"db": "pii", "key": "ijms19082214"}], "notes": [], "created": "2018-08-28T07:00:46.756Z", "modified": "2024-04-03T14:49:15.948Z"}]}