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Carraro, Gianni; Langerman, Justin; Sabri, Shan; Lorenzana, Zareeb; Purkayastha, Arunima; Zhang, Guangzhu; Konda, Bindu; Aros, Cody J; Calvert, Ben A; Szymaniak, Aleks; Wilson, Emily; Mulligan, Michael; Bhatt, Priyanka; Lu, Junjie; Vijayaraj, Preethi; Yao, Changfu; Shia, David W; Lund, Andrew J; Israely, Edo; Rickabaugh, Tammy M; Ernst, Jason; Mense, Martin; Randell, Scott H; Vladar, Eszter K; Ryan, Amy L; Plath, Kathrin; Mahoney, John E; Stripp, Barry R; Gomperts, Brigitte N
Nature medicine, 05/2021, Letnik: 27, Številka: 5Journal Article
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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JCR | SNIP | JCR | SNIP | JCR | SNIP | JCR | SNIP |
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Povezave do osebnih bibliografij avtorjev | Povezave do podatkov o raziskovalcih v sistemu SICRIS |
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Vir: Osebne bibliografije
in: SICRIS
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