The authors demonstrated a different expression of several genes associated with hepatic fibrogenesis including, matrix metalloproteinases, collagens and chemokines in CFLD versus the control patients, particularly decreased expression in tissue remodelling genes including tissue inhibitor of metalloproteinase-1 (TIMP-1) and plasminogen activator inhibitor-1 (PAI-1, up to 25-fold) (32).These studies show a possible genetic predisposition, independent of the CFTR gene, in the pathogenesis of CFLD but further data are needed to add details about this issue. Here, SERPINE1 is linked to cystic fibrosis-related liver disease.