Emerging Roles of Matricellular Proteins in Systemic Sclerosis.
biomarkers/therapeutics
extracellular matrix
fibrosis
matricellular proteins
myofibroblasts
systemic sclerosis
Journal
International journal of molecular sciences
ISSN: 1422-0067
Titre abrégé: Int J Mol Sci
Pays: Switzerland
ID NLM: 101092791
Informations de publication
Date de publication:
06 Jul 2020
06 Jul 2020
Historique:
received:
31
05
2020
revised:
11
06
2020
accepted:
13
06
2020
entrez:
10
7
2020
pubmed:
10
7
2020
medline:
9
3
2021
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 32640520
pii: ijms21134776
doi: 10.3390/ijms21134776
pmc: PMC7369781
pii:
doi:
Substances chimiques
Extracellular Matrix Proteins
0
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
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