Processing of Neutrophil α-Defensins Does Not Rely on Serine Proteases In Vivo
Fig 6
Serine protease activity is not required for HNP processing in vivo.
Peripheral blood neutrophils from two patients suffering from Papillon–Lefèvre syndrome (PLS). (A) Neutrophil elastase (NE) and proteinase 3 (PR3) activity was measured as absorbance (Abs) following degradation of methoxysuc-AAPV-p-nitroanilide. Assay from patient 1 is shown and is representative of both patients. (B) Cathepsin G (CG) activity was measured as absorbance following degradation of N-suc-AAPF-p-nitroanilide. Assay from patient 1 is shown and is representative of both patients. (C) Western blotting of NE, CG, and PR3 in two PLS patients and controls. (D) Western blotting of HNP and β-actin in two PLS patients and controls.