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Abbreviations ABC ATP-binding cassette CF cystic fibrosis CFTR cystic fibrosis transmembrane conductance regulator MBL2 mannose-binding lectin 2 PH prolyl hydroxylase TGFβ transforming growth factor β TIMP tissue inhibitor of matrix metalloproteinase TNFα tumor necrosis factor α UDCA ursodeoxycholic acid Cystic…
Abbreviations AAT alpha-1 antitrypsin ER endoplasmic reticulum PAS periodic acid–Schiff Pi protease inhibitor Alpha-1 antitrypsin (AAT) is the most abundant protease inhibitor and the second most abundant protein in plasma. It is produced predominantly by the liver and in smaller…