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Here, we report the CRISPR\/Cas9-mediated generation of CF rabbits, a model with a relatively long lifespan and affordable maintenance and care costs. CF rabbits supplemented solely with oral osmotic laxative had a median survival of approximately 40 days and died of gastrointestinal disease, but therapeutic regimens directed toward restoring gastrointestinal transit extended median survival to approximately 80 days. Surrogate markers of exocrine pancreas disorders were found in CF rabbits with declining health. CFTR expression patterns in WT rabbit airways mimicked humans, with widespread distribution in nasal respiratory and olfactory epithelia, as well as proximal and distal lower airways. CF rabbits exhibited human CF\u2013like abnormalities in the bioelectric properties of the nasal and tracheal epithelia. No spontaneous respiratory disease was detected in young CF rabbits. However, abnormal phenotypes were observed in surviving 1-year-old CF rabbits as compared with WT littermates, and these were especially evident in the nasal respiratory and olfactory epithelium. The CF rabbit model may serve as a useful tool for understanding gut and lung CF pathogenesis and for the practical development of CF therapeutics.<\/jats:p>","DOI":"10.1172\/jci.insight.139813","type":"journal-article","created":{"date-parts":[[2020,11,24]],"date-time":"2020-11-24T13:31:53Z","timestamp":1606224713000},"source":"Crossref","is-referenced-by-count":35,"title":["Phenotypes of CF rabbits generated by CRISPR\/Cas9-mediated disruption of the CFTR gene"],"prefix":"10.1172","volume":"6","author":[{"given":"Jie","family":"Xu","sequence":"first","affiliation":[{"name":"Center for Advanced Models for Translational Sciences and Therapeutics, University of Michigan (UM) Medical Center, Ann Arbor, Michigan, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8751-3631","authenticated-orcid":true,"given":"Alessandra","family":"Livraghi-Butrico","sequence":"additional","affiliation":[{"name":"Marsico Lung Institute, University of North 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