{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Arellano G"],"funding":["Amy and David Fulton Foundation","Thomas and Deige McLaughlin Foundation","Rottering Family Foundation","NIAID NIH HHS","NINDS NIH HHS","National Institutes of Health","Johnnie Walkers MS Foundation","NIH HHS","Crammer Family Foundation"],"pagination":["1344-1361"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC10994540"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["147(4)"],"pubmed_abstract":["Neuromyelitis optica spectrum disorder (NMOSD) is a CNS autoimmune inflammatory disease mediated by T helper 17 (Th17) and antibody responses to the water channel protein, aquaporin 4 (AQP4), and associated with astrocytopathy, demyelination and axonal loss. Knowledge about disease pathogenesis is limited and the search for new therapies impeded by the absence of a reliable animal model. In our work, we determined that NMOSD is characterized by decreased IFN-γ receptor signalling and that IFN-γ depletion in AQP4201-220-immunized C57BL/6 mice results in severe clinical disease resembling human NMOSD. Pathologically, the disease causes autoimmune astrocytic and CNS injury secondary to cellular and humoral inflammation. Immunologically, the absence of IFN-γ allows for increased expression of "],"journal":["Brain : a journal of neurology"],"pubmed_title":["Interferon-γ controls aquaporin 4-specific Th17 and B cells in neuromyelitis optica spectrum disorder."],"pmcid":["PMC10994540"],"funding_grant_id":["R21AI151438","R21 AI151438","R01 NS034939","R01 NS099334"],"pubmed_authors":["Kaschke K","Podojil JR","Miller SD","Popko B","Balabanov R","Neef T","Arellano G","Cogswell AC","Chen Y","Primer G","Wills S","Joy G","Loda E"],"additional_accession":[]},"is_claimable":false,"name":"Interferon-γ controls aquaporin 4-specific Th17 and B cells in neuromyelitis optica spectrum disorder.","description":"Neuromyelitis optica spectrum disorder (NMOSD) is a CNS autoimmune inflammatory disease mediated by T helper 17 (Th17) and antibody responses to the water channel protein, aquaporin 4 (AQP4), and associated with astrocytopathy, demyelination and axonal loss. Knowledge about disease pathogenesis is limited and the search for new therapies impeded by the absence of a reliable animal model. In our work, we determined that NMOSD is characterized by decreased IFN-γ receptor signalling and that IFN-γ depletion in AQP4201-220-immunized C57BL/6 mice results in severe clinical disease resembling human NMOSD. Pathologically, the disease causes autoimmune astrocytic and CNS injury secondary to cellular and humoral inflammation. Immunologically, the absence of IFN-γ allows for increased expression of ","dates":{"release":"2024-01-01T00:00:00Z","publication":"2024 Apr","modification":"2026-06-01T03:15:52.671Z","creation":"2025-04-19T09:58:17.834Z"},"accession":"S-EPMC10994540","cross_references":{"pubmed":["37931066"],"doi":["10.1093/brain/awad373"]}}