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Título del libro: Contemporary Approaches To Idiopathic Pulmonary Fibrosis
Título del capítulo: Epithelial biology in idiopathic pulmonary fibrosis

Autores UNAM:
ANNIE PARDO CEMO;
Autores externos:

Idioma:
Inglés
Año de publicación:
2015
Resumen:

A growing body of evidence strongly suggests that epithelial cells play a key role in the initiation and perpetuation of idiopathic pulmonary fibrosis (IPF). The main environmental risk factors for the initiation and/or progression of IPF that affect the alveolar epithelium are the exposure to cigarette-smoke, viral infections and microaspiration (gastro-oesophageal reflux). In familial IPF as well as in monogenetic diseases, such as Hemansky-Pudlak syndrome and Nieman-Pick disease, alveolar epithelial cells also to play a crucial role in the fibrotic response. A subset of patients with familial IPF shows heterozygous mutations in either the hTERT or the hTERC gene of the telomerase with abnormal shortening of telomeres that affect the alveolar epithelial cells likely impairing their regenerative capacity. In IPF, epithelial cells are highly active and secrete almost all the profibrotic mediators that participate in the formation of the fibroblastic/myofibroblastic foci and the subsequent tissue remodeling. Relentless recapitulation of embryological pathways, such as Wnt/-catenin pathway and Sonic Hedgehog pathway, that affect both epithelial cells and fibroblasts, seems to be implicated in the pathogenesis of IPF. © 2015 Future Medicine Ltd. All rights reserved.


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