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Role of matrix metalloproteinases (MMPs) in cystic fibrosis

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Matrix Metalloproteinases in Tissue Remodelling and Inflammation

Part of the book series: Progress in Inflammation Research ((PIR))

Abstract

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.

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Author information

Authors and Affiliations

  1. Respiratory Medicine Department, Pontchaillou Hospital, 35000, Rennes, France

    Stéphane Jouneau, Guillaume Léveiller, Graziella Brinchault, Chantal Belleguic & Benoît Desrues

  2. INSERM U620, Faculté de Pharmacie, Université de Rennes 1, 2 avenue du Professeur Léon Bernard, 35000, Rennes, France

    Stéphane Jouneau & Vincent Lagente

  3. Pathology Department, Pontchaillou Hospital, 35000, Rennes, France

    Sylvie Caulet-Maugendre

Authors
  1. Stéphane Jouneau
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  2. Guillaume Léveiller
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  3. Sylvie Caulet-Maugendre
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  4. Graziella Brinchault
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  5. Chantal Belleguic
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  6. Benoît Desrues
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  7. Vincent Lagente
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Editor information

Editors and Affiliations

  1. INSERM U620 Faculté des Sciences Pharmaceutiques et Biologique, Université de Rennes 1, Avenue du Professeur Léon Bernard 2, 35043, Rennes CX, France

    Vincent Lagente  & Elisabeth Boichot  & 

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© 2008 Birkhäuser Verlag Basel/Switzerland

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Jouneau, S. et al. (2008). Role of matrix metalloproteinases (MMPs) in cystic fibrosis. In: Lagente, V., Boichot, E. (eds) Matrix Metalloproteinases in Tissue Remodelling and Inflammation. Progress in Inflammation Research. Birkhäuser Basel. https://doi.org/10.1007/978-3-7643-8585-9_5

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