| Literature DB >> 33958799 |
Gianni Carraro1, Justin Langerman2, Shan Sabri2, Zareeb Lorenzana3,4, Arunima Purkayastha5, Guangzhu Zhang1, Bindu Konda1, Cody J Aros5,6,7, Ben A Calvert3, Aleks Szymaniak8, Emily Wilson8, Michael Mulligan8, Priyanka Bhatt8, Junjie Lu8, Preethi Vijayaraj5, Changfu Yao1, David W Shia5,6,7, Andrew J Lund5,6, Edo Israely1, Tammy M Rickabaugh5, Jason Ernst2,9,10, Martin Mense8, Scott H Randell11, Eszter K Vladar12, Amy L Ryan3,4, Kathrin Plath13,14,15, John E Mahoney16, Barry R Stripp17, Brigitte N Gomperts18,19,20,21.
Abstract
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.Entities:
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Year: 2021 PMID： 33958799 PMCID： PMC9009537 DOI： 10.1038/s41591-021-01332-7
Source DB: PubMed Journal: Nat Med ISSN： 1078-8956 Impact factor: 53.440