Abstract
BackgroundCombined pulmonary fibrosis and emphysema (CPFE) is characterized by both emphysema of the upper zone and diffuse parenchymal lung disease with fibrosis of the lower zone of the lung on chest computed tomography. The aim of this study was to investigate the mechanism of CPFE regarding gene expressions by comparing the results of microarray sequences between fibrotic and emphysematous lesions in the lungs of CPFE patients.ResultsThe expression profiles of the fibrotic and emphysematous lesions were remarkably different in terms of function. Genes related to the immune system, structural constituents of the cytoskeleton, and cellular adhesion were overexpressed in fibrotic lesions, while genes associated with the cellular fraction, cell membrane structures, vascular growth and biology, second-messenger-mediated signaling, and lung development (all processes that contribute to the destruction and repair of cells, vessels, and the lung) were overexpressed in emphysematous lesions.ConclusionsThe differences in gene expression were detected in fibrotic and emphysematous lesions in CPFE patients. We propose that the development of coexisting fibrotic and emphysematous lesions in CPFE is implemented by these different patterns of gene expressions.
Highlights
Combined pulmonary fibrosis and emphysema (CPFE) is characterized by both emphysema of the upper zone and diffuse parenchymal lung disease with fibrosis of the lower zone of the lung on chest computed tomography
The set of genes overexpressed in the emphysematous lesions were annotated as associated with cell membrane structures, vascular growth and biology, second-messenger-mediated signaling, and lung development – all processes that contribute to the destruction and repair of cells, vessels, and the lung
Different from other findings in which the results of gene expression in pulmonary fibrosis were resulted from the comparisons of the gene expression datasets between fibrous tissues and healthy lung tissues [13] or hypersensitivity pneumonitis lung tissues [15], we found that gene members of the structural constituent of cytoskeleton annotation cluster were overexpressed in CPFE fibrotic lesions versus emphysematous lesions
Summary
Combined pulmonary fibrosis and emphysema (CPFE) is characterized by both emphysema of the upper zone and diffuse parenchymal lung disease with fibrosis of the lower zone of the lung on chest computed tomography. The aim of this study was to investigate the mechanism of CPFE regarding gene expressions by comparing the results of microarray sequences between fibrotic and emphysematous lesions in the lungs of CPFE patients. One of the most demonstrably clinical features of combined pulmonary fibrosis and emphysema is that emphysema of the upper zones and diffuse parenchymal lung disease with fibrosis of the lower zones of the lungs are both presented on chest computed tomography [1]. We hypothesized that coexistent fibrosis and emphysema are programmed by differential gene expressions in the corresponding lesions in the lungs of smokers susceptible to CPFE. Given the importance of genetic susceptibility in understanding the etiology and pathogenesis of CPFE, we performed the whole-genome microarray to sequence the gene expression profiling on fibrotic and emphysematous tissues sampled from three patients with CPFE to identify genes distinguishably expressed in fibrotic lesions and emphysematous lesions in lung tissues of patients with CPFE
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