Other studies have demonstrated that while the overall abundance of matrix proteins in CF lung tissue may not differ significantly from controls, the diversity and specific composition of matrix proteins are altered, with the majority (> 90%) of individual matrix proteins expressed at lower levels in CF compared to control lung54 as a result from a chronic protease-antiprotease imbalance in CF airways where high level of neutrophil elastase and low levels of protease inhibitors are observed, leading to excessive breakdown of extracellular matrix components55,56. Here, ELANE is linked to cystic fibrosis.