{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,25]],"date-time":"2026-10-25T20:25:10Z","timestamp":1792959910618,"version":"4.5.0"},"reference-count":29,"publisher":"American Association for the Advancement of Science (AAAS)","issue":"5897","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Science"],"published-print":{"date-parts":[[2008,9,26]]},"abstract":"<jats:p>\n                    Almost two decades after\n                    <jats:italic>CFTR<\/jats:italic>\n                    was identified as the gene responsible for cystic fibrosis (CF), we still lack answers to many questions about the pathogenesis of the disease, and it remains incurable. Mice with a disrupted\n                    <jats:italic>CFTR<\/jats:italic>\n                    gene have greatly facilitated CF studies, but the mutant mice do not develop the characteristic manifestations of human CF, including abnormalities of the pancreas, lung, intestine, liver, and other organs. Because pigs share many anatomical and physiological features with humans, we generated pigs with a targeted disruption of both\n                    <jats:italic>CFTR<\/jats:italic>\n                    alleles. Newborn pigs lacking CFTR exhibited defective chloride transport and developed meconium ileus, exocrine pancreatic destruction, and focal biliary cirrhosis, replicating abnormalities seen in newborn humans with CF. The pig model may provide opportunities to address persistent questions about CF pathogenesis and accelerate discovery of strategies for prevention and treatment.\n                  <\/jats:p>","DOI":"10.1126\/science.1163600","type":"journal-article","created":{"date-parts":[[2008,9,26]],"date-time":"2008-09-26T08:25:38Z","timestamp":1222417538000},"page":"1837-1841","source":"Crossref","is-referenced-by-count":690,"title":["Disruption of the\n                    <i>CFTR<\/i>\n                    Gene Produces a Model of Cystic Fibrosis in Newborn Pigs"],"prefix":"10.1126","volume":"321","author":[{"given":"Christopher S.","family":"Rogers","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA."},{"name":"Department of Pathology, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA."},{"name":"Howard Hughes Medical Institute (HHMI), Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA."},{"name":"Department of Pediatrics, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA."},{"name":"Department of Surgery, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David A.","family":"Stoltz","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA."},{"name":"Department of Pathology, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA."},{"name":"Howard Hughes Medical Institute (HHMI), Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA."},{"name":"Department of Pediatrics, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA."},{"name":"Department of Surgery, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David K.","family":"Meyerholz","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA."},{"name":"Department of Pathology, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA."},{"name":"Howard Hughes Medical Institute (HHMI), Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA."},{"name":"Department of Pediatrics, Roy J. and Lucille A. 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Care Med."},{"key":"e_1_3_2_26_2","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.20294"},{"key":"e_1_3_2_27_2","doi-asserted-by":"publisher","DOI":"10.1023\/A:1011080229374"},{"key":"e_1_3_2_28_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200206-627OC"},{"key":"e_1_3_2_29_2","doi-asserted-by":"publisher","DOI":"10.1165\/ajrcmb.23.2.4214"},{"key":"e_1_3_2_30_2","unstructured":"We thank A. Arias E. Bagnall J. Bartlett B. Bauer T. Bohnert E. Burnight K. Dobbs C. Dohrn L. Dowell L. Gakhar D. Guilliams Y. Hao S. C. Isom S. Jones S. Korte B. Kussman J. Launspach M. Linville E. Mahan T. Mayhew C. McHughes K. Munson M. Parker C. Randak S. Ramachandran J. Ross A. Small L. Spate D. Vermeer E. Walters and J. Whyte for excellent assistance. We thank our patients with CF for inspiration and we also thank Cystic Fibrosis Foundation Therapeutics and R. Bridges for the gift of GlyH-101. This work was supported by the National Heart Lung and Blood Institute (grant HL51670) the National Institute of Diabetes and Digestive and Kidney Diseases (grant DK54759) Food for the 21st Century the Cystic Fibrosis Foundation and HHMI. C.S.R. was supported by NIH training grant HL07638. D.A.S. is a Parker B. Francis Fellow and was supported by the National Institute of Allergy and Infectious Diseases (grant AI076671). M.J.W. is an Investigator of the HHMI. C.S.R. R.S.P. M.J.W. and the University of Iowa Foundation have applied for a patent related to the work reported in this paper and C.S.R. and M.J.W. are founders of Exemplar Genetics a company that is licensing materials and technology related to this work. C.S.R. is currently Director of Research and Development at Exemplar Genetics."}],"container-title":["Science"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.science.org\/doi\/pdf\/10.1126\/science.1163600","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,10]],"date-time":"2024-01-10T04:52:43Z","timestamp":1704862363000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.science.org\/doi\/10.1126\/science.1163600"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,9,26]]},"references-count":29,"journal-issue":{"issue":"5897","published-print":{"date-parts":[[2008,9,26]]}},"alternative-id":["10.1126\/science.1163600"],"URL":"https:\/\/doi.org\/10.1126\/science.1163600","relation":{"has-review":[{"id-type":"doi","id":"10.3410\/f.1123954.581549","asserted-by":"object"},{"id-type":"doi","id":"10.3410\/f.1123954.580115","asserted-by":"object"},{"id-type":"doi","id":"10.3410\/f.1123954.581054","asserted-by":"object"}]},"ISSN":["0036-8075","1095-9203"],"issn-type":[{"value":"0036-8075","type":"print"},{"value":"1095-9203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2008,9,26]]}}}