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Microarray profiling reveals suppressed interferon stimulated gene program in fibroblasts from scleroderma-associated
Gisela E Lindahl1, Carmel Jw Stock, Xu Shi-Wen
1Interstitial Lung Disease Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College London, Emmanuel Kaye Building, 1B Manresa Road, London SW3 6LR, UK. g.lindahl@imperial.ac.uk
Respiratory Research
|August 7, 2013
Summary
Researchers found suppressed interferon-stimulated genes in lung fibroblasts from patients with systemic sclerosis-associated interstitial lung disease. This suppressed gene program may drive pulmonary fibrosis, indicating a new target for treatment.
Area of Science:
- Pulmonary Medicine
- Fibrosis Research
- Molecular Biology
Background:
- Interstitial lung disease (ILD) is a significant cause of mortality in systemic sclerosis (SSc).
- Pulmonary fibrosis in SSc involves fibroblast proliferation and extracellular matrix accumulation.
- Understanding SSc lung fibroblast gene expression is crucial for deciphering fibrotic processes.
Purpose of the Study:
- To characterize the gene expression profiles of fibroblasts from SSc-associated ILD lungs.
- To compare these profiles with control lung fibroblasts and idiopathic pulmonary fibrosis (IPF) fibroblasts.
- To identify molecular pathways contributing to the fibrotic phenotype in SSc-ILD.
Main Methods:
- Gene expression profiling of pulmonary fibroblasts using Affymetrix microarrays.
- Comparison between SSc-ILD (n=8), IPF (n=3), and control lung fibroblasts (n=10).
- Differential gene expression analysis validated by qRT-PCR and/or western blot.
Main Results:
- Identified 843 differentially expressed genes in SSc-ILD and/or IPF fibroblasts versus controls.
- Observed increased TGF-β response signatures and myofibroblast markers.
- Found uniformly suppressed expression of interferon-stimulated genes (ISGs) in fibrotic fibroblasts, including STAT1 and CXCL10.
Conclusions:
- A strongly suppressed interferon-stimulated gene program exists in fibroblasts from fibrotic lungs.
- Repressed ISG expression may be a key factor in the profibrotic fibroblast phenotype.
- This finding highlights a potential therapeutic target for pulmonary fibrosis.
