ADAMTS1 and cystic fibrosis-related liver disease: Many of the proteins found up-regulated in CFLD have previously been described in fibrotic processes of different organs and/or liver fibrosis such as GM-CSF, ADAMTS-1, IP-10, PDGF-AB/-BB, TGF-ß1 or Activin A. Another 9 serum proteins were found to be at least 2-fold decreased in our serum proteome analysis.