Emerging Roles of Matricellular Proteins in Systemic Sclerosis.

Abstract:

: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.

journal_name

Int J Mol Sci

authors

Feng D,Gerarduzzi C

doi

10.3390/ijms21134776

subject

Has Abstract

pub_date

2020-07-06 00:00:00

issue

13

issn

1422-0067

pii

ijms21134776

journal_volume

21

pub_type

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