In addition, the interaction between PR3 and PGRN also likely has wider impact than in COPD, through a further role in inflammatory conditions involving PGRN, including lipopolysaccharide-induced acute lung injury, dermatitis and inflammatory arthritis (in murine models), as well as a reported genetic link between loss-of-function mutations in PGRN and the development of neurodegenerative disease [99–104]. Here, GRN is linked to neurodegenerative disease.