학술논문

Emerging Roles of Matricellular Proteins in Systemic Sclerosis.
Document Type
Article
Source
International Journal of Molecular Sciences. Jul2020, Vol. 21 Issue 13, p4776-4776. 1p.
Subject
*SYSTEMIC scleroderma
*EXTRACELLULAR matrix proteins
*PROTEINS
*PROTEIN expression
*VIRAL nonstructural proteins
Language
ISSN
1661-6596
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. [ABSTRACT FROM AUTHOR]