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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...

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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
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cited_by cdi_FETCH-LOGICAL-c456t-fe4d437306f29a022b577b0c9b5caea48d9a6acf050a42b641ed9e7b110f54ca3
cites cdi_FETCH-LOGICAL-c456t-fe4d437306f29a022b577b0c9b5caea48d9a6acf050a42b641ed9e7b110f54ca3
container_end_page eaba1983
container_issue 28
container_start_page eaba1983
container_title Science advances
container_volume 6
creator 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
description 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.
doi_str_mv 10.1126/sciadv.aba1983
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subjects Developmental Biology
Diseases and Disorders
Endothelial Cells
Humans
Idiopathic Pulmonary Fibrosis - genetics
Lung
Pulmonary Disease, Chronic Obstructive
RNA-Seq
SciAdv r-articles
title Single-cell RNA-seq reveals ectopic and aberrant lung-resident cell populations in idiopathic pulmonary fibrosis
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