AQP4-specific T cells determine lesion localization in the CNS in a model of NMOSD.

ABSTRACT: Neuromyelitis optica spectrum disorder (NMOSD) is a paradigmatic autoimmune disease of the central nervous system (CNS), in which the water channel protein Aquaporin-4 (AQP4) is targeted by a self-reactive immune response. While the immunopathology of human NMOSD is largely dependent on antibodies to astrocytic AQP4, the role of AQP4-specific T cells for the localization and quality of NMOSD lesions in the CNS is not known. Only recently, we established that thymic B cells express and present AQP4 in the context of MHC class II molecules to purge the naive T cell receptor repertoire of AQP4-specific clones. Here, we exploited this finding to investigate the lesion localization in the CNS of B cell conditional AQP4-deficient (Aqp4^ΔB) mice, which harbor AQP4-specific precursors in

SUBMITTER: Afzali AM 

PROVIDER: S-EPMC11817536 | biostudies-literature | 2025 Feb

REPOSITORIES: biostudies-literature