ELANE and chronic granulomatous disease: However, experiments using NE-deficient models and nonselective NE inhibitors [86, 144–146], along with data from neutrophils in patients with chronic granulomatous disease (CDG), which prevents respiratory burst [17, 140], and Papillon‒Lefèvre syndrome (PLS), which is characterized by mutations in CTSC that prevent NSP activation [147, 148], support the role of NE in NETosis.