Role of matrix metalloproteinases (MMPs) in cystic fibrosis
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Cystic fibrosis (CF) is the most frequent hereditary lethal disease in Caucasians. It is the consequence of a mutation in a chloride channel called CFTR. This defective channel leads to viscous secretions in all exocrine glands and therefore destruction and fibrosis of these organs. The main point of CF is the lung disease with inflammation and infection leading towards remodelling. There are only symptomatic treatments. Matrix metalloproteinases (MMPs) play several roles in CF development. Indeed, MMPs are involved in the regulation of CFTR channel. The alveolar levels of MMPs in CF patients are increased compared to controls with active form and lead to an imbalance between proteases and anti-proteases. MMPs are enhanced in sputum and plasma in severe CF patients. MMPs also have a role in regeneration of human CF airway surface epithelium and differentiation. MMPs could also interfere with the aerosolised medication. Together, these data exhibit the major role of MMPs in CF.
KeywordsCystic Fibrosis Cystic Fibrosis Transmembrane Conductance Regulator Force Vital Capacity Respir Crit Cystic Fibrosis Lung
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