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Revista del Instituto Nacional de Enfermedades Respiratorias

Print version ISSN 0187-7585

Abstract

RAMOS ABRAHAM, CARLOS; BECERRIL BERROCAL, CARINA; CISNEROS LIRA, JOSÉ G.  and  MONTANO RAMIREZ, MARTHA. EL MIOFIBROBLASTO, UNA CÉLULA MULTIFUNCIONAL EN LA PATOLOGÍA PULMONAR. Rev. Inst. Nal. Enf. Resp. Mex. [online]. 2004, vol.17, n.3, pp.215-231. ISSN 0187-7585.

Myofibroblasts are a fibroblast subpopulation with a phenotype similar to smooth muscle cells, since they express the cytoskeletal a-smooth muscle actin (α-SMA); however, in different organs, they show some phenotypical differences. Their physiology is similar in the different tissues and organs. Based on their extensive spectrum of synthesis and secretion of molecules such as cytokines, interleukins, chemokines, growth factors, lipids, diverse physiological mediators, molecules of the extracelullar matrix, MMPs and TIMPs, they play a very important role during embryogenesis, organogenesis, inflammation, repair and wound healing, besides being fundamental in the processes of regeneration and repair (fibrosis) that occur in the different organs. In the case of the respiratory system, the myofibroblasts are as important in the air ways as in the lung, mainly participating in the diverse pathogenic processes; whether in pathologies with a derivative pattern such as emphysema, or in diseases with a fibrogenic pattern with excessive synthesis of the diverse components of the extracellular matrix, as occurs in pulmonary fibrosis and asthma. Myofibroblasts are especially important in the different forms of pulmonary fibrosis whether idiopathic or of known cause. Idiopathic fibrosis, as asthma, seems to be essentially restricted to areas of tissue adjacent to damaged alveolar-epithelial areas, where a physiopathogenic relation of type II neumocyte-myofibroblast-fibroblast exists. On the other hand, myofibroblasts are mostly derived from fibroblast transdifferentiation by TGF-β1 stimulation.

Keywords : Fibroblast; myofibroblast; pulmonary fibrosis; extracellular matrix; a-smooth muscle actin.

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