{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T05:33:02Z","timestamp":1780551182887,"version":"3.54.1"},"reference-count":102,"publisher":"American Society for Clinical Investigation","issue":"13","license":[{"start":{"date-parts":[[2024,7,1]],"date-time":"2024-07-01T00:00:00Z","timestamp":1719792000000},"content-version":"vor","delay-in-days":48,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003725","name":"National Research Foundation of Korea","doi-asserted-by":"crossref","award":["2021R1A6A3A14039132,2021R1A6A3A-14039681"],"award-info":[{"award-number":["2021R1A6A3A14039132,2021R1A6A3A-14039681"]}],"id":[{"id":"10.13039\/501100003725","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"crossref","award":["R01-AA029124,HL091842,HL152960"],"award-info":[{"award-number":["R01-AA029124,HL091842,HL152960"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100016616","name":"Cystic Fibrosis Research","doi-asserted-by":"crossref","award":["STOLTZ19R0"],"award-info":[{"award-number":["STOLTZ19R0"]}],"id":[{"id":"10.13039\/100016616","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,7,1]]},"abstract":"<jats:p>\n                    Mutations in the cystic fibrosis transmembrane conductance regulator (\n                    <jats:italic>CFTR<\/jats:italic>\n                    ) gene cause cystic fibrosis (CF), a multiorgan disease that is characterized by diverse metabolic defects. However, other than specific\n                    <jats:italic>CFTR<\/jats:italic>\n                    mutations, the factors that influence disease progression and severity remain poorly understood. Aberrant metabolite levels have been reported, but whether\n                    <jats:italic>CFTR<\/jats:italic>\n                    loss itself or secondary abnormalities (infection, inflammation, malnutrition, and various treatments) drive metabolic defects is uncertain. Here, we implemented comprehensive arteriovenous metabolomics in newborn CF pigs, and the results revealed\n                    <jats:italic>CFTR<\/jats:italic>\n                    as a bona fide regulator of metabolism.\n                    <jats:italic>CFTR<\/jats:italic>\n                    loss impaired metabolite exchange across organs, including disruption of lung uptake of fatty acids, yet enhancement of uptake of arachidonic acid, a precursor of proinflammatory cytokines.\n                    <jats:italic>CFTR<\/jats:italic>\n                    loss also impaired kidney reabsorption of amino acids and lactate and abolished renal glucose homeostasis. These and additional unexpected metabolic defects prior to disease manifestations reveal a fundamental role for\n                    <jats:italic>CFTR<\/jats:italic>\n                    in controlling multiorgan metabolism. Such discovery informs a basic understanding of CF, provides a foundation for future investigation, and has implications for developing therapies targeting only a single tissue.\n                  <\/jats:p>","DOI":"10.1172\/jci174500","type":"journal-article","created":{"date-parts":[[2024,6,30]],"date-time":"2024-06-30T14:00:35Z","timestamp":1719756035000},"source":"Crossref","is-referenced-by-count":11,"title":["Arteriovenous metabolomics in pigs reveals CFTR regulation of metabolism in multiple organs"],"prefix":"10.1172","volume":"134","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5848-7972","authenticated-orcid":true,"given":"Hosung","family":"Bae","sequence":"first","affiliation":[{"name":"Department of Biological Chemistry, University of California \u2013 Irvine, Irvine, California, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bo Ram","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Pappajohn Biomedical Institute, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA."},{"name":"Howard Hughes Medical Institute, University of Iowa, Iowa City, Iowa, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sunhee","family":"Jung","sequence":"additional","affiliation":[{"name":"Department of Biological Chemistry, University of California \u2013 Irvine, Irvine, California, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0245-5848","authenticated-orcid":true,"given":"Johnny","family":"Le","sequence":"additional","affiliation":[{"name":"Department of Biological Chemistry, University of California \u2013 Irvine, Irvine, California, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dana","family":"van der Heide","sequence":"additional","affiliation":[{"name":"Department of Surgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3013-1218","authenticated-orcid":true,"given":"Wenjie","family":"Yu","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Pappajohn Biomedical Institute, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA."},{"name":"Howard Hughes Medical Institute, University of Iowa, Iowa City, Iowa, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sang Hee","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Biological Chemistry, University of California \u2013 Irvine, Irvine, California, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brieanna M.","family":"Hilkin","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Pappajohn Biomedical Institute, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4069-9683","authenticated-orcid":true,"given":"Nicholas D.","family":"Gansemer","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Pappajohn Biomedical Institute, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda S.","family":"Powers","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Pappajohn Biomedical Institute, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Taekyung","family":"Kang","sequence":"additional","affiliation":[{"name":"Department of Biological Chemistry, University of California \u2013 Irvine, Irvine, California, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1552-3253","authenticated-orcid":true,"given":"David K.","family":"Meyerholz","sequence":"additional","affiliation":[{"name":"Department of Pathology, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6192-0017","authenticated-orcid":true,"given":"Victor L.","family":"Schuster","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Albert Einstein College of Medicine, Bronx, New York, New York, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6651-4213","authenticated-orcid":true,"given":"Cholsoon","family":"Jang","sequence":"additional","affiliation":[{"name":"Department of Biological Chemistry, University of California \u2013 Irvine, Irvine, California, USA."},{"name":"Center for Complex Biological Systems and"},{"name":"Center for Epigenetics and Metabolism, University of California \u2013 Irvine, Irvine, California, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1646-6206","authenticated-orcid":true,"given":"Michael J.","family":"Welsh","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Pappajohn Biomedical Institute, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA."},{"name":"Howard Hughes Medical Institute, University of Iowa, Iowa City, Iowa, USA."},{"name":"Department of Molecular Physiology and Biophysics, Pappajohn Biomedical Institute, Roy J. and Lucille A. Carver College of Medicine University of Iowa, Iowa City, Iowa, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"232","published-online":{"date-parts":[[2024,5,14]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1152\/physrev.1999.79.1.S3"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1038\/nrdp.2015.10"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(16)00576-6"},{"key":"B4","unstructured":"Welsh MJ, Ramsey BW, Accurso F, Cutting GR. Cystic Fibrosis. In: Valle DL, Antonarakis S, Ballabio A, Beaudet AL, Mitchell GA, eds. The Online Metabolic and Molecular Bases of Inherited Disease. McGraw-Hill Education; 2019. 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