Journal
NATURE MEDICINE
Volume 15, Issue 6, Pages 623-625Publisher
NATURE PUBLISHING GROUP
DOI: 10.1038/nm.1959
Keywords
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Funding
- Deutsche Forschungsgemeinschaft [SFB 571]
- US National Institutes of Health [AI053194]
- Susan G. Komen for the Cure Foundation
- Alexander von Humboldt Foundation
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Small-vessel vasculitis (SVV) is a chronic autoinflammatory condition linked to antineutrophil cytoplasm autoantibodies (ANCAs). Here we show that chromatin fibers, so-called neutrophil extracellular traps (NETs), are released by ANCA-stimulated neutrophils and contain the targeted autoantigens proteinase-3 (PR3) and myeloperoxidase (MPO). Deposition of NETs in inflamed kidneys and circulating MPO-DNA complexes suggest that NET formation triggers vasculitis and promotes the autoimmune response against neutrophil components in individuals with SVV.
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