show Abstracthide AbstractAnti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is autoimmune diseases characterized by multiple organ injury due to the pathogenic autoantibodies. The impaired regulation of neutrophil extracellular traps (NETs) is involved in the pathogenesis of this diseases. Based on the concept that NETs might contribute to the diverse clinical and pathological findings, we focused on the mechanism of ANCA stimulated NET formation as a signaling of the programmed necrosis. Cyclophilin D (CypD) is a mitochondrial matrix protein and regulates necrosis via the opening of mPTP. Wild type or CypD knockout neutrophils treated with mMPO-IgG were subjected to RNA-sequencing to investigate the involvement of CypD dependent necrosis in ANCA induced NETs.