[HTML][HTML] Complement receptor Mac-1 is an adaptor for NB1 (CD177)-mediated PR3-ANCA neutrophil activation
The glycosylphosphatidylinositol (GPI)-anchored neutrophil-specific receptor NB1 (CD177)
presents the autoantigen proteinase 3 (PR3) on the membrane of a neutrophil subset. PR3-
ANCA-activated neutrophils participate in small-vessel vasculitis. Since NB1 lacks an
intracellular domain, we characterized components of the NB1 signaling complex that are
pivotal for neutrophil activation. PR3-ANCA resulted in degranulation and superoxide
production in the mNB1 pos/PR3 high neutrophils, but not in the mNB1 neg/PR3 low subset …
presents the autoantigen proteinase 3 (PR3) on the membrane of a neutrophil subset. PR3-
ANCA-activated neutrophils participate in small-vessel vasculitis. Since NB1 lacks an
intracellular domain, we characterized components of the NB1 signaling complex that are
pivotal for neutrophil activation. PR3-ANCA resulted in degranulation and superoxide
production in the mNB1 pos/PR3 high neutrophils, but not in the mNB1 neg/PR3 low subset …