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A Single-Cell Atlas of Large and Small Airways at Birth in a Porcine Model of Cystic Fibrosis
Journal article   Open access   Peer reviewed

A Single-Cell Atlas of Large and Small Airways at Birth in a Porcine Model of Cystic Fibrosis

Andrew L Thurman, Xiaopeng Li, Raul Villacreses, Wenjie Yu, Huiyu Gong, Steven E Mather, Guillermo S Romano-Ibarra, David K Meyerholz, David A Stoltz, Michael J Welsh, …
American journal of respiratory cell and molecular biology, Vol.66(6), pp.612-622
03/02/2022
DOI: 10.1165/rcmb.2021-0499OC
PMCID: PMC9163647
PMID: 35235762
url
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9163647View
Published (Version of record) Open Access

Abstract

Lack of CFTR affects the transcriptome, composition, and function of large and small airway epithelia in people with advanced cystic fibrosis (CF); however, whether lack of CFTR causes cell-intrinsic abnormalities present at birth vs. inflammation-dependent abnormalities is unclear. We performed a single cell RNA-seq census of micro-dissected small airways from newborn CF pigs, which recapitulate CF host defense defects and pathology over time. Lack of CFTR minimally affected the transcriptome of large and small airways at birth, suggesting that infection and inflammation drive transcriptomic abnormalities in advanced CF. Importantly, common small airway epithelial cell types expressed a markedly different transcriptome than corresponding large airway cell types. Quantitative immunohistochemistry and electrophysiology of small airway epithelia demonstrated basal cells that reach the apical surface and a water and ion transport advantage. This single cell atlas highlights the archetypal nature of airway epithelial cells with location-dependent gene expression and function.
airway epithelia single cell rna-seq sus scrofa small airway

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