title: Role of matrix metalloproteinases in the pathogenesis of idiopathic pulmonary fibrosis.
abstract: Idiopathic pulmonary fibrosis (IPF) is a progressive and devastating lung disorder of unknown origin, with very poor prognosis and no effective treatment. The disease is characterized by abnormal activation of alveolar epithelial cells, which secrete numerous mediators involved in the expansion of the fibroblast population, its differentiation to myofibroblasts, and in the exaggerated accumulation of extracellular matrix provoking the loss of lung architecture. Among the excessively produced mediators are several matrix metalloproteases (MMPs) which may contribute to modify the lung microenvironment by various mechanisms. Thus, these enzymes can not only degrade all the components of the extracellular matrix, but they are also able to release, cleave and activate a wide range of growth factors, cytokines, chemokines and cell surface receptors affecting numerous cell functions including adhesion, proliferation, differentiation, recruiting and transmigration, and apoptosis. Therefore, dysregulated expression of MMPs may have profound impact on the biopathological mechanisms implicated in the development of IPF. This review focuses on the current and emerging evidence regarding the role of MMPs on the fibrotic processes in IPF as well as in mouse models of lung fibrosis.
authors.affiliation: Facultad de Ciencias, Universidad Nacional Autónoma de México, México, DF, Mexico. apardos@unam.mx.
authors.forename: Annie
authors.initials: A
authors.lastname: Pardo
authors.affiliation: Facultad de Ciencias, Universidad Nacional Autónoma de México, México, DF, Mexico.
authors.forename: Sandra
authors.initials: S
authors.lastname: Cabrera
authors.affiliation: Facultad de Ciencias, Universidad Nacional Autónoma de México, México, DF, Mexico.
authors.forename: Mariel
authors.initials: M
authors.lastname: Maldonado
authors.affiliation: Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, México, DF, Mexico.
authors.forename: Moisés
authors.initials: M
authors.lastname: Selman
mesh_terms: D000818:Animals; D005109:Extracellular Matrix / Q000201:enzymology* / Q000473:pathology; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000201:enzymology* / Q000473:pathology*; D008168:Lung / Q000201:enzymology* / Q000473:pathology*; D020782:Matrix Metalloproteinases / Q000378:metabolism*; D008954:Models, Biological
chemical_list: D020782:Matrix Metalloproteinases