Endothelial to Mesenchymal Transition (EndoMT) in the Pathogenesis of Human Fibrotic Diseases
Fibrotic diseases encompass a wide spectrum of clinical entities including systemic fibrotic diseases such as systemic sclerosis, sclerodermatous graft versus host disease, nephrogenic systemic fibrosis, and IgG4-associated sclerosing disease, as well as numerous organ-specific disorders including r...
Main Authors: | , , |
---|---|
Format: | Article |
Language: | English |
Published: |
MDPI AG
2016-04-01
|
Series: | Journal of Clinical Medicine |
Subjects: | |
Online Access: | http://www.mdpi.com/2077-0383/5/4/45 |
id |
doaj-8cbdfb9135494386964a304c65f4349a |
---|---|
record_format |
Article |
spelling |
doaj-8cbdfb9135494386964a304c65f4349a2020-11-24T20:57:01ZengMDPI AGJournal of Clinical Medicine2077-03832016-04-01544510.3390/jcm5040045jcm5040045Endothelial to Mesenchymal Transition (EndoMT) in the Pathogenesis of Human Fibrotic DiseasesSonsoles Piera-Velazquez0Fabian A. Mendoza1Sergio A. Jimenez2Jefferson Institute of Molecular Medicine, Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, 233 S. 10th Street, Suite 509 BLSB, Philadelphia, PA 19107, USARheumatology Division, Department of Medicine, Thomas Jefferson University, 233 S. 10th Street, Suite 509 BLSB, Philadelphia, PA 19107, USAJefferson Institute of Molecular Medicine, Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, 233 S. 10th Street, Suite 509 BLSB, Philadelphia, PA 19107, USAFibrotic diseases encompass a wide spectrum of clinical entities including systemic fibrotic diseases such as systemic sclerosis, sclerodermatous graft versus host disease, nephrogenic systemic fibrosis, and IgG4-associated sclerosing disease, as well as numerous organ-specific disorders including radiation-induced fibrosis, and cardiac, pulmonary, liver, and kidney fibrosis. Although their causative mechanisms are quite diverse, these diseases share the common feature of an uncontrolled and progressive accumulation of fibrous tissue macromolecules in affected organs leading to their dysfunction and ultimate failure. The pathogenesis of fibrotic diseases is complex and despite extensive investigation has remained elusive. Numerous studies have identified myofibroblasts as the cells responsible for the establishment and progression of the fibrotic process. Tissue myofibroblasts in fibrotic diseases originate from several sources including quiescent tissue fibroblasts, circulating CD34+ fibrocytes, and the phenotypic conversion of various cell types including epithelial and endothelial cells into activated myofibroblasts. However, the role of the phenotypic transition of endothelial cells into mesenchymal cells (Endothelial to Mesenchymal Transition or EndoMT) in the pathogenesis of fibrotic disorders has not been fully elucidated. Here, we review the evidence supporting EndoMT’s contribution to human fibrotic disease pathogenesis.http://www.mdpi.com/2077-0383/5/4/45Endothelial Mesenchymal TransitionEndoMTfibrosisfibrotic diseasessystemic sclerosisidiopathic pulmonary fibrosisendothelial cellmyofibroblastcollagenextracellular matrixtransforming growth factor-β |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Sonsoles Piera-Velazquez Fabian A. Mendoza Sergio A. Jimenez |
spellingShingle |
Sonsoles Piera-Velazquez Fabian A. Mendoza Sergio A. Jimenez Endothelial to Mesenchymal Transition (EndoMT) in the Pathogenesis of Human Fibrotic Diseases Journal of Clinical Medicine Endothelial Mesenchymal Transition EndoMT fibrosis fibrotic diseases systemic sclerosis idiopathic pulmonary fibrosis endothelial cell myofibroblast collagen extracellular matrix transforming growth factor-β |
author_facet |
Sonsoles Piera-Velazquez Fabian A. Mendoza Sergio A. Jimenez |
author_sort |
Sonsoles Piera-Velazquez |
title |
Endothelial to Mesenchymal Transition (EndoMT) in the Pathogenesis of Human Fibrotic Diseases |
title_short |
Endothelial to Mesenchymal Transition (EndoMT) in the Pathogenesis of Human Fibrotic Diseases |
title_full |
Endothelial to Mesenchymal Transition (EndoMT) in the Pathogenesis of Human Fibrotic Diseases |
title_fullStr |
Endothelial to Mesenchymal Transition (EndoMT) in the Pathogenesis of Human Fibrotic Diseases |
title_full_unstemmed |
Endothelial to Mesenchymal Transition (EndoMT) in the Pathogenesis of Human Fibrotic Diseases |
title_sort |
endothelial to mesenchymal transition (endomt) in the pathogenesis of human fibrotic diseases |
publisher |
MDPI AG |
series |
Journal of Clinical Medicine |
issn |
2077-0383 |
publishDate |
2016-04-01 |
description |
Fibrotic diseases encompass a wide spectrum of clinical entities including systemic fibrotic diseases such as systemic sclerosis, sclerodermatous graft versus host disease, nephrogenic systemic fibrosis, and IgG4-associated sclerosing disease, as well as numerous organ-specific disorders including radiation-induced fibrosis, and cardiac, pulmonary, liver, and kidney fibrosis. Although their causative mechanisms are quite diverse, these diseases share the common feature of an uncontrolled and progressive accumulation of fibrous tissue macromolecules in affected organs leading to their dysfunction and ultimate failure. The pathogenesis of fibrotic diseases is complex and despite extensive investigation has remained elusive. Numerous studies have identified myofibroblasts as the cells responsible for the establishment and progression of the fibrotic process. Tissue myofibroblasts in fibrotic diseases originate from several sources including quiescent tissue fibroblasts, circulating CD34+ fibrocytes, and the phenotypic conversion of various cell types including epithelial and endothelial cells into activated myofibroblasts. However, the role of the phenotypic transition of endothelial cells into mesenchymal cells (Endothelial to Mesenchymal Transition or EndoMT) in the pathogenesis of fibrotic disorders has not been fully elucidated. Here, we review the evidence supporting EndoMT’s contribution to human fibrotic disease pathogenesis. |
topic |
Endothelial Mesenchymal Transition EndoMT fibrosis fibrotic diseases systemic sclerosis idiopathic pulmonary fibrosis endothelial cell myofibroblast collagen extracellular matrix transforming growth factor-β |
url |
http://www.mdpi.com/2077-0383/5/4/45 |
work_keys_str_mv |
AT sonsolespieravelazquez endothelialtomesenchymaltransitionendomtinthepathogenesisofhumanfibroticdiseases AT fabianamendoza endothelialtomesenchymaltransitionendomtinthepathogenesisofhumanfibroticdiseases AT sergioajimenez endothelialtomesenchymaltransitionendomtinthepathogenesisofhumanfibroticdiseases |
_version_ |
1716789026237710336 |