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Emerging Roles of Matricellular Proteins in Systemic Sclerosis
Systemic sclerosis is a rare chronic heterogenous disease that involves inflammation and vasculopathy, and converges in end-stage development of multisystem tissue fibrosis. The loss of tight spatial distribution and temporal expression of proteins in the extracellular matrix (ECM) leads to progress...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7369781/ https://www.ncbi.nlm.nih.gov/pubmed/32640520 http://dx.doi.org/10.3390/ijms21134776 |
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author | Feng, Daniel Gerarduzzi, Casimiro |
author_facet | Feng, Daniel Gerarduzzi, Casimiro |
author_sort | Feng, Daniel |
collection | PubMed |
description | Systemic sclerosis is a rare chronic heterogenous disease that involves inflammation and vasculopathy, and converges in end-stage development of multisystem tissue fibrosis. The loss of tight spatial distribution and temporal expression of proteins in the extracellular matrix (ECM) leads to progressive organ stiffening, which is a hallmark of fibrotic disease. A group of nonstructural matrix proteins, known as matricellular proteins (MCPs) are implicated in dysregulated processes that drive fibrosis such as ECM remodeling and various cellular behaviors. Accordingly, MCPs have been described in the context of fibrosis in sclerosis (SSc) as predictive disease biomarkers and regulators of ECM synthesis, with promising therapeutic potential. In this present review, an informative summary of major MCPs is presented highlighting their clear correlations to SSc- fibrosis. |
format | Online Article Text |
id | pubmed-7369781 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-73697812020-07-21 Emerging Roles of Matricellular Proteins in Systemic Sclerosis Feng, Daniel Gerarduzzi, Casimiro Int J Mol Sci Review Systemic sclerosis is a rare chronic heterogenous disease that involves inflammation and vasculopathy, and converges in end-stage development of multisystem tissue fibrosis. The loss of tight spatial distribution and temporal expression of proteins in the extracellular matrix (ECM) leads to progressive organ stiffening, which is a hallmark of fibrotic disease. A group of nonstructural matrix proteins, known as matricellular proteins (MCPs) are implicated in dysregulated processes that drive fibrosis such as ECM remodeling and various cellular behaviors. Accordingly, MCPs have been described in the context of fibrosis in sclerosis (SSc) as predictive disease biomarkers and regulators of ECM synthesis, with promising therapeutic potential. In this present review, an informative summary of major MCPs is presented highlighting their clear correlations to SSc- fibrosis. MDPI 2020-07-06 /pmc/articles/PMC7369781/ /pubmed/32640520 http://dx.doi.org/10.3390/ijms21134776 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Feng, Daniel Gerarduzzi, Casimiro Emerging Roles of Matricellular Proteins in Systemic Sclerosis |
title | Emerging Roles of Matricellular Proteins in Systemic Sclerosis |
title_full | Emerging Roles of Matricellular Proteins in Systemic Sclerosis |
title_fullStr | Emerging Roles of Matricellular Proteins in Systemic Sclerosis |
title_full_unstemmed | Emerging Roles of Matricellular Proteins in Systemic Sclerosis |
title_short | Emerging Roles of Matricellular Proteins in Systemic Sclerosis |
title_sort | emerging roles of matricellular proteins in systemic sclerosis |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7369781/ https://www.ncbi.nlm.nih.gov/pubmed/32640520 http://dx.doi.org/10.3390/ijms21134776 |
work_keys_str_mv | AT fengdaniel emergingrolesofmatricellularproteinsinsystemicsclerosis AT gerarduzzicasimiro emergingrolesofmatricellularproteinsinsystemicsclerosis |