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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Bibliographic Details
Published in:International journal of molecular sciences 2020-07, Vol.21 (13), p.4776
Main Authors: Feng, Daniel, Gerarduzzi, Casimiro
Format: Article
Language:English
Subjects:
Animals
biomarkers/therapeutics
Biomechanics
Cell adhesion & migration
Collagen
Extracellular matrix
Extracellular Matrix - metabolism
Extracellular Matrix Proteins - metabolism
Fibroblasts
Fibrosis
Growth factors
Heparan sulfate
Humans
matricellular proteins
myofibroblasts
Proteins
Regulators
Review
Scleroderma
Scleroderma, Systemic - metabolism
Scleroderma, Systemic - pathology
Senescence
Signal transduction
Spatial distribution
Stiffening
Systemic sclerosis
Vascular diseases
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Summary: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.
ISSN:1422-0067
1661-6596
1422-0067
DOI:10.3390/ijms21134776