{"doi":"10.1101/2020.05.01.072876","title":"A molecular atlas of proximal airway identifies subsets of known airway cell types revealing details of the unique molecular pathogenesis of Cystic Fibrosis","abstract":"Introduction/Abstract Cystic fibrosis (CF) is a lethal autosomal recessive disorder that afflicts in excess of 70,000 people globally. 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 significant determinant of morbidity and mortality. In this study we report results from a multi-institute consortium in which single cell transcriptomics were applied to define disease-related changes to the proximal airway of CF donors (n=19) undergoing transplantation for end-stage lung disease compared to the proximal airway of previously healthy lung donors (n=19). We found that all major airway epithelial cell types were conserved between control and CF donors. Disease-dependent differences were observed, including an overabundance of epithelial cells transitioning to specialized ciliated and secretory cell subtypes coupled with an unexpected decrease in cycling basal cells. This study developed a molecular atlas of the proximal airway epithelium that will provide insights for the development of new targeted therapies for CF airway disease.","journal":"bioRxiv (Cold Spring Harbor Laboratory)","year":2020,"id":123629,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":3,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9266,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2020-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":253275,"name":"Justin Langerman","orcid":null,"position":1,"is_corresponding":false},{"id":252009,"name":"Shan Sabri","orcid":"0000-0002-0885-6159","position":2,"is_corresponding":false},{"id":566954,"name":"Zareeb Lorenzana","orcid":"0000-0002-4735-280X","position":3,"is_corresponding":false},{"id":286904,"name":"Arunima Purkayastha","orcid":null,"position":4,"is_corresponding":false},{"id":284478,"name":"Bindu Konda","orcid":"0000-0001-5949-922X","position":5,"is_corresponding":false},{"id":315955,"name":"Cody J. 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Rickabaugh","orcid":null,"position":16,"is_corresponding":false},{"id":275321,"name":"Martin Mense","orcid":"0000-0003-0280-9789","position":17,"is_corresponding":false},{"id":72632,"name":"Scott H. Randell","orcid":"0000-0001-5351-2841","position":18,"is_corresponding":false},{"id":240438,"name":"Eszter K. Vladar","orcid":"0000-0002-4160-8894","position":19,"is_corresponding":false},{"id":467674,"name":"Amy L. Ryan","orcid":"0000-0003-1363-905X","position":20,"is_corresponding":false},{"id":252018,"name":"Kathrin Plath","orcid":"0000-0001-7796-3372","position":21,"is_corresponding":false},{"id":274045,"name":"John E. Mahoney","orcid":"0000-0001-9414-9110","position":22,"is_corresponding":false},{"id":230901,"name":"Barry R. Stripp","orcid":"0000-0003-4348-2137","position":23,"is_corresponding":false},{"id":284480,"name":"Brigitte N. 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