Single-cell RNA-seq reveals ectopic and aberrant lung-resident cell populations in idiopathic pulmonary fibrosis

We provide a single-cell atlas of idiopathic pulmonary fibrosis (IPF), a fatal interstitial lung disease, by profiling 312,928 cells from 32 IPF, 28 smoker and nonsmoker controls, and 18 chronic obstructive pulmonary disease (COPD) lungs. Among epithelial cells enriched in IPF, we identify a previou...

Full description

Saved in:
Bibliographic Details
Published in:Science advances 2020-07, Vol.6 (28), p.eaba1983-eaba1983
Main Authors: Adams, Taylor S, Schupp, Jonas C, Poli, Sergio, Ayaub, Ehab A, Neumark, Nir, Ahangari, Farida, Chu, Sarah G, Raby, Benjamin A, DeIuliis, Giuseppe, Januszyk, Michael, Duan, Qiaonan, Arnett, Heather A, Siddiqui, Asim, Washko, George R, Homer, Robert, Yan, Xiting, Rosas, Ivan O, Kaminski, Naftali
Format: Article
Language:English
Subjects:
Developmental Biology
Diseases and Disorders
Endothelial Cells
Humans
Idiopathic Pulmonary Fibrosis - genetics
Lung
Pulmonary Disease, Chronic Obstructive
RNA-Seq
SciAdv r-articles
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:We provide a single-cell atlas of idiopathic pulmonary fibrosis (IPF), a fatal interstitial lung disease, by profiling 312,928 cells from 32 IPF, 28 smoker and nonsmoker controls, and 18 chronic obstructive pulmonary disease (COPD) lungs. Among epithelial cells enriched in IPF, we identify a previously unidentified population of aberrant basaloid cells that coexpress basal epithelial, mesenchymal, senescence, and developmental markers and are located at the edge of myofibroblast foci in the IPF lung. Among vascular endothelial cells, we identify an ectopically expanded cell population transcriptomically identical to bronchial restricted vascular endothelial cells in IPF. We confirm the presence of both populations by immunohistochemistry and independent datasets. Among stromal cells, we identify IPF myofibroblasts and invasive fibroblasts with partially overlapping cells in control and COPD lungs. Last, we confirm previous findings of profibrotic macrophage populations in the IPF lung. Our comprehensive catalog reveals the complexity and diversity of aberrant cellular populations in IPF.
ISSN:2375-2548
2375-2548
DOI:10.1126/sciadv.aba1983