Transcriptional analysis of cystic fibrosis airways at single-cell resolution reveals altered epithelial cell states and composition

Gianni Carraro, Justin Langerman, Shan Sabri, Zareeb Lorenzana, Arunima Purkayastha, Guangzhu Zhang, Bindu Konda, Cody J Aros, Ben A Calvert, Aleks Szymaniak, Emily Wilson, Michael Mulligan, Priyanka Bhatt, Junjie Lu, Preethi Vijayaraj, Changfu Yao, David W Shia, Andrew J Lund, Edo Israely, Tammy M Rickabaugh, Jason Ernst, Martin Mense, Scott H Randell, Eszter K Vladar, Amy L Ryan, Kathrin Plath, John E Mahoney, Barry R Stripp, Brigitte N Gomperts
Nature Medicine 2021 May 6
Cystic fibrosis (CF) is a lethal autosomal recessive disorder that afflicts more than 70,000 people. People with CF experience multi-organ dysfunction resulting from aberrant electrolyte transport across polarized epithelia due to mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. CF-related lung disease is by far the most important determinant of morbidity and mortality. Here we report results from a multi-institute consortium in which single-cell transcriptomics were applied to define disease-related changes by comparing the proximal airway of CF donors (n = 19) undergoing transplantation for end-stage lung disease with that of previously healthy lung donors (n = 19). Disease-dependent differences observed include an overabundance of epithelial cells transitioning to specialized ciliated and secretory cell subsets coupled with an unexpected decrease in cycling basal cells. Our study yields a molecular atlas of the proximal airway epithelium that will provide insights for the development of new targeted therapies for CF airway disease.

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