Role of CD248 as a potential severity marker in idiopathic pulmonary fibrosis

CD248 or Endosialin is a transmembrane molecule expressed in stromal cells binding to extracellular matrix (ECM) components. It has been previously implicated in kidney fibrosis, rheumatoid arthritis as well as in tumour-stromal interactions. This study investigates the role of CD248 in the pathogen...

Full description

Saved in:
Bibliographic Details
Published in:BMC pulmonary medicine 2016-04, Vol.16 (1), p.51-51, Article 51
Main Authors: Bartis, Domokos, Crowley, Louise E, D'Souza, Vijay K, Borthwick, Lee, Fisher, Andrew J, Croft, Adam P, Pongrácz, Judit E, Thompson, Richard, Langman, Gerald, Buckley, Christopher D, Thickett, David R
Format: Article
Language:English
Subjects:
Adult
Aged
Analysis
Antigens, CD - biosynthesis
Antigens, CD - genetics
Antigens, Neoplasm - biosynthesis
Antigens, Neoplasm - genetics
Biological markers
Biomarkers - metabolism
Biopsy
Care and treatment
Cell Proliferation
Cells, Cultured
Complications and side effects
Female
Fibroblasts - metabolism
Fibroblasts - pathology
Flow Cytometry
Gene Expression Regulation
Humans
Idiopathic Pulmonary Fibrosis - diagnosis
Idiopathic Pulmonary Fibrosis - genetics
Idiopathic Pulmonary Fibrosis - metabolism
Immunohistochemistry
Lung - metabolism
Lung - pathology
Male
Middle Aged
Pulmonary fibrosis
Pulmonology
Real-Time Polymerase Chain Reaction
Retrospective Studies
RNA - genetics
Severity of Illness Index
Signal Transduction
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:CD248 or Endosialin is a transmembrane molecule expressed in stromal cells binding to extracellular matrix (ECM) components. It has been previously implicated in kidney fibrosis, rheumatoid arthritis as well as in tumour-stromal interactions. This study investigates the role of CD248 in the pathogenesis of fibrotic diseases in Idiopathic Pulmonary Fibrosis (IPF). CD248 quantitative immunohistochemistry (IHC) was performed on lung samples from 22 IPF patients and its expression was assayed in cultured pulmonary fibroblasts and epithelial cells. Effects of CD248 silencing was evaluated on fibroblast proliferation and myofibroblast differentiation. IHC revealed strong CD248 expression in mesenchymal cells of normal lung structures such as pleura and adventitia but not in epithelium. Fibrotic areas showed markedly stronger staining than unaffected lung tissue. The extent of CD248 staining showed a significant negative correlation to lung function parameters FEV1, FVC, TLC, and TLCO (r2 > 0 · 35, p 
ISSN:1471-2466
1471-2466
DOI:10.1186/s12890-016-0211-7