Inflammatory transcriptomic signatures in a human cellular NMOSD model reveal upregulation of NF-κB and IL6 pathways.

ABSTRACT: Neuromyelitis optica spectrum disorder (NMOSD) is a rare neurological autoimmune disease caused by autoantibodies targeting the astrocytic water channel aquaporin-4 (AQP4). Binding to AQP4 initiates the activation of innate immune components, especially the complement system. Both in-vivo and in-vitro models have been developed to study the molecular pathophysiology of NMOSD. Our study aimed to characterize the molecular response of four human cell lines (AQP4-ECFP expressing U-87MG glioblastoma cells, U-87MG expressing only ECFP, HEK293 cells expressing AQP4-EmGFP, and human primary astrocytes) to a treatment with AQP4 antibody E5415A and human complement. Complement-dependent cytotoxicity was induced by this treatment in AQP4-expressing cells by the terminal complement pathway. Transcrip

SUBMITTER: Brandl S 

PROVIDER: S-EPMC12686536 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature