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Among PLS patient families, clinically asymptomatic heterozygous carriers demonstrated DPP1 activity reduced to only 13%–47% of controls, suggesting that PLS-like symptoms may only emerge when DPP1 inhibition exceeds 80%–90% ( 3.2 Role of NSPs in inflammatory lung diseases In inflammatory lung diseases including NCFBE, COPD, and CF, neutrophil-mediated inflammatory responses constitute the core pathological mechanism (Voynow and Shinbashi, 2021