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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...

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Detalles Bibliográficos
Autores principales: Feng, Daniel, Gerarduzzi, Casimiro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
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.
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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
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