Cargando…
Fibrosis in connective tissue disease: the role of the myofibroblast and fibroblast-epithelial cell interactions
Fibrosis, characterized by excessive extracellular matrix accumulation, is a common feature of many connective tissue diseases, notably scleroderma (systemic sclerosis). Experimental studies suggest that a complex network of intercellular interactions involving endothelial cells, epithelial cells, f...
Autores principales: | , , |
---|---|
Formato: | Texto |
Lenguaje: | English |
Publicado: |
BioMed Central|1
2007
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2072888/ https://www.ncbi.nlm.nih.gov/pubmed/17767742 http://dx.doi.org/10.1186/ar2188 |
_version_ | 1782137800381956096 |
---|---|
author | Krieg, Thomas Abraham, David Lafyatis, Robert |
author_facet | Krieg, Thomas Abraham, David Lafyatis, Robert |
author_sort | Krieg, Thomas |
collection | PubMed |
description | Fibrosis, characterized by excessive extracellular matrix accumulation, is a common feature of many connective tissue diseases, notably scleroderma (systemic sclerosis). Experimental studies suggest that a complex network of intercellular interactions involving endothelial cells, epithelial cells, fibroblasts and immune cells, using an array of molecular mediators, drives the pathogenic events that lead to fibrosis. Transforming growth factor-β and endothelin-1, which are part of a cytokine hierarchy with connective tissue growth factor, are key mediators of fibrogenesis and are primarily responsible for the differentiation of fibroblasts toward a myofibroblast phenotype. The tight skin mouse (Tsk-1) model of cutaneous fibrosis suggests that numerous other genes may also be important. |
format | Text |
id | pubmed-2072888 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2007 |
publisher | BioMed Central|1 |
record_format | MEDLINE/PubMed |
spelling | pubmed-20728882007-11-10 Fibrosis in connective tissue disease: the role of the myofibroblast and fibroblast-epithelial cell interactions Krieg, Thomas Abraham, David Lafyatis, Robert Arthritis Res Ther Review Fibrosis, characterized by excessive extracellular matrix accumulation, is a common feature of many connective tissue diseases, notably scleroderma (systemic sclerosis). Experimental studies suggest that a complex network of intercellular interactions involving endothelial cells, epithelial cells, fibroblasts and immune cells, using an array of molecular mediators, drives the pathogenic events that lead to fibrosis. Transforming growth factor-β and endothelin-1, which are part of a cytokine hierarchy with connective tissue growth factor, are key mediators of fibrogenesis and are primarily responsible for the differentiation of fibroblasts toward a myofibroblast phenotype. The tight skin mouse (Tsk-1) model of cutaneous fibrosis suggests that numerous other genes may also be important. BioMed Central|1 2007 2007-08-15 /pmc/articles/PMC2072888/ /pubmed/17767742 http://dx.doi.org/10.1186/ar2188 Text en Copyright © 2007 BioMed Central Ltd |
spellingShingle | Review Krieg, Thomas Abraham, David Lafyatis, Robert Fibrosis in connective tissue disease: the role of the myofibroblast and fibroblast-epithelial cell interactions |
title | Fibrosis in connective tissue disease: the role of the myofibroblast and fibroblast-epithelial cell interactions |
title_full | Fibrosis in connective tissue disease: the role of the myofibroblast and fibroblast-epithelial cell interactions |
title_fullStr | Fibrosis in connective tissue disease: the role of the myofibroblast and fibroblast-epithelial cell interactions |
title_full_unstemmed | Fibrosis in connective tissue disease: the role of the myofibroblast and fibroblast-epithelial cell interactions |
title_short | Fibrosis in connective tissue disease: the role of the myofibroblast and fibroblast-epithelial cell interactions |
title_sort | fibrosis in connective tissue disease: the role of the myofibroblast and fibroblast-epithelial cell interactions |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2072888/ https://www.ncbi.nlm.nih.gov/pubmed/17767742 http://dx.doi.org/10.1186/ar2188 |
work_keys_str_mv | AT kriegthomas fibrosisinconnectivetissuediseasetheroleofthemyofibroblastandfibroblastepithelialcellinteractions AT abrahamdavid fibrosisinconnectivetissuediseasetheroleofthemyofibroblastandfibroblastepithelialcellinteractions AT lafyatisrobert fibrosisinconnectivetissuediseasetheroleofthemyofibroblastandfibroblastepithelialcellinteractions |