Proteinase 3 (PR3) is the essential antigenic target of antineutrophil cytoplasm antibodies in Wegener’s granulomatosis (now called granulomatosis with polyangiitis). There are many structural and functional similarities between neutrophil elastase (NE) and PR3. The development of highly sensitive substrates and inhibitors of human PR3 and the creation of PR3-selective single knockout mice led to the identification of nonredundant roles of PR3 in cell death induction via procaspase-3 activation in cell cultures and in mouse models.
Proteinase 3: the odd one out that became an autoantigen.
Martin KR, Witko-Sarsat V.
J Leukoc Biol. 2017 Sep;102(3):689-698
Korkmaz B, Lesner A, Guarino C, Wysocka M, Kellenberger C, Watier H, Specks U, Gauthier F, Jenne DE.
Pharmacol Rev. 2016 Jul;68(3):603-30.
