Related Experiment Video
Updated: Sep 27, 2025

Ex Vivo Corneal Organ Culture Model for Wound Healing Studies
Published on: February 15, 2019
PDGF/PDGFR: A Possible Molecular Target in Scleroderma Fibrosis
Chiara Paolini1, Silvia Agarbati1, Devis Benfaremo1,2
1Department of Clinical and Molecular Sciences, Università Politecnica delle Marche, 60126 Ancona, Italy.
Abstract:
Systemic sclerosis (SSc) is a clinically heterogeneous disorder of the connective tissue characterized by vascular alterations, immune/inflammatory manifestations, and organ fibrosis. SSc pathogenesis is complex and still poorly understood. Therefore, effective therapies are lacking and remain nonspecific and limited to disease symptoms. In the last few years, many molecular and cellular mediators of SSc fibrosis have been described, providing new potential options for targeted therapies. In this review: (i) we focused on the PDGF/PDGFR pathway as key signaling molecules in the development of tissue fibrosis; (ii) we highlighted the possible role of stimulatory anti-PDGFRα autoantibodies in the pathogenesis of SSc; (iii) we reported the most promising PDGF/PDGFR targeting therapies.
Insights
Systemic sclerosis (SSc) involves complex fibrosis. This review explores the PDGF/PDGFR pathway
Area of Science:
- Connective tissue disorders
- Immunology
- Fibrosis research
Background:
- Systemic sclerosis (SSc) is a heterogeneous connective tissue disease.
- Pathogenesis involves vascular, immune, and fibrotic changes.
- Current SSc therapies are limited and nonspecific.
Purpose of the Study:
- To review the role of the PDGF/PDGFR pathway in SSc fibrosis.
- To discuss the potential involvement of anti-PDGFRα autoantibodies in SSc.
- To highlight emerging PDGF/PDGFR-targeting therapies for SSc.
Main Methods:
- Literature review focusing on PDGF/PDGFR signaling in fibrosis.
- Analysis of studies investigating anti-PDGFRα autoantibodies in SSc.
- Compilation of data on PDGF/PDGFR-targeted therapeutic strategies.
Main Results:
- The PDGF/PDGFR pathway is a key mediator of tissue fibrosis in SSc.
- Stimulatory anti-PDGFRα autoantibodies may contribute to SSc pathogenesis.
- Several PDGF/PDGFR-targeting therapies show promise for SSc treatment.
Conclusions:
- Targeting the PDGF/PDGFR pathway offers a promising therapeutic avenue for SSc.
- Further research into anti-PDGFRα autoantibodies could elucidate SSc mechanisms.
- Developing specific PDGF/PDGFR inhibitors may lead to effective SSc treatments.
More Related Videos
08:09Vasodilation of Isolated Vessels and the Isolation of the Extracellular Matrix of Tight-skin Mice
Published on: March 24, 2017
11:38Visualization and Quantification of TGFβ/BMP/SMAD Signaling under Different Fluid Shear Stress Conditions using Proximity-Ligation-Assay
Published on: September 14, 2021
Related Concept Videos
TGF - β Signaling Pathway
Regulation of Angiogenesis and Blood Supply