{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T14:32:28Z","timestamp":1790087548383,"version":"4.0.1"},"reference-count":53,"publisher":"American Physiological Society","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American Journal of Physiology-Gastrointestinal and Liver Physiology"],"published-print":{"date-parts":[[2004,6]]},"abstract":"<jats:p>The CFTR null mouse [cystic fibrosis (CF) mouse] has a severe intestinal phenotype that serves as a model for CF-related growth deficiency, meconium ileus, and distal intestinal obstructive syndrome. DNA microarray analysis was used to investigate gene expression in the CF mouse small intestine. Sixty-one genes exhibited a statistically significant twofold or greater increase in expression, and 98 genes were downregulated twofold or greater. Of the upregulated genes, most were associated with inflammation and included markers for cells of the innate immune system (mast cells and neutrophils) and for acute-phase genes (serum amyloid A and complement factors). The downregulated genes include 10 cytochrome P-450 genes; several are involved in lipid metabolism, and several are involved in various transport processes. Confirmation by quantitative RT-PCR showed gene expression was significantly increased for mast cell protease 2 (27-fold), hematopoietic cell transcript 1 (17-fold), serum amyloid A3 (2.9-fold), suppressor of cytokine signaling 3 (2.0-fold), leucine-rich \u03b1<jats:sub>2<\/jats:sub>-glycoprotein (21-fold), resistin-like molecule-\u03b2 (49-fold), and Muclin (2.5-fold) and was significantly decreased for cytochrome P-450 4a10 (28-fold) and cubilin (114-fold). Immune cell infiltration was confirmed histologically by staining for mast cells and neutrophils. These data demonstrate that the CF intestine exhibits an inflammatory state with upregulation of components of the innate immune system.<\/jats:p>","DOI":"10.1152\/ajpgi.00473.2003","type":"journal-article","created":{"date-parts":[[2004,5,7]],"date-time":"2004-05-07T18:53:20Z","timestamp":1083956000000},"page":"G1032-G1041","source":"Crossref","is-referenced-by-count":126,"title":["Inflammation of the cystic fibrosis mouse small intestine"],"prefix":"10.1152","volume":"286","author":[{"given":"Oxana","family":"Norkina","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simran","family":"Kaur","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donna","family":"Ziemer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert C.","family":"De Lisle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"24","reference":[{"key":"R1","doi-asserted-by":"publisher","DOI":"10.1097\/00005176-199607000-00009"},{"key":"R2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.physiol.62.1.535"},{"key":"R3","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0185(199603)244:3<327::AID-AR5>3.0.CO;2-V"},{"key":"R4","doi-asserted-by":"publisher","DOI":"10.1385\/CRIAI:23:1:005"},{"key":"R5","unstructured":"Clarke LL, Stien X, and Walker NM.Intestinal bicarbonate secretion in cystic fibrosis mice.JOP2: 263\u2013267, 2001."},{"key":"R6","doi-asserted-by":"publisher","DOI":"10.1016\/S0304-4165(02)00491-9"},{"key":"R7","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-3024.2002.00457.x"},{"key":"R8","doi-asserted-by":"publisher","DOI":"10.1002\/jcb.240560315"},{"key":"R9","doi-asserted-by":"publisher","DOI":"10.1152\/ajpgi.2001.281.4.G899"},{"key":"R10","doi-asserted-by":"publisher","DOI":"10.1053\/gast.1997.v113.pm9247472"},{"key":"R11","doi-asserted-by":"publisher","DOI":"10.1152\/ajpgi.1998.275.2.G219"},{"key":"R12","doi-asserted-by":"crossref","unstructured":"DiScipio RG.Ultrastructures and interactions of complement factors H and I.J Immunol149: 2592\u20132599, 1992.","DOI":"10.4049\/jimmunol.149.8.2592"},{"key":"R13","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.1995.269.5.L625"},{"key":"R14","doi-asserted-by":"publisher","DOI":"10.1016\/S1074-7613(00)80591-9"},{"key":"R15","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2003-08-2852"},{"key":"R16","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1997.tb14836.x"},{"key":"R17","unstructured":"Goldsby RA, Kindt TJ, Osborne BA, and Kuby J.Immunology. New York: Freeman, 2003."},{"key":"R18","doi-asserted-by":"publisher","DOI":"10.1053\/gast.2002.37068"},{"key":"R19","doi-asserted-by":"publisher","DOI":"10.1152\/ajpgi.1999.277.1.G167"},{"key":"R20","doi-asserted-by":"publisher","DOI":"10.1097\/00006676-199009000-00011"},{"key":"R21","doi-asserted-by":"publisher","DOI":"10.1016\/j.gastro.2003.07.009"},{"key":"R22","doi-asserted-by":"publisher","DOI":"10.1097\/00005176-200109000-00006"},{"key":"R23","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/19.15.4046"},{"key":"R24","doi-asserted-by":"publisher","DOI":"10.1126\/science.291.5505.881"},{"key":"R25","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.95.17.10078"},{"key":"R26","doi-asserted-by":"publisher","DOI":"10.1016\/S0014-5793(03)00217-5"},{"key":"R27","doi-asserted-by":"publisher","DOI":"10.1292\/jvms.64.419"},{"key":"R28","doi-asserted-by":"publisher","DOI":"10.1177\/39.10.1682362"},{"key":"R29","unstructured":"Lebenthal E, Lerner A, and Rolston DDK.The pancreas in cystic fibrosis. In:The Pancreas: Biology, Pathobiology, and Disease, edited by Go VLW, DiMagno EP, Gardner JD, Lebenthal E, Reber HA, and Scheele GA. New York: Raven, 1993, p. 1041\u20131081."},{"key":"R30","doi-asserted-by":"publisher","DOI":"10.1093\/oxfordjournals.bmb.a072581"},{"key":"R31","doi-asserted-by":"publisher","DOI":"10.1038\/ng0997-32"},{"key":"R32","doi-asserted-by":"publisher","DOI":"10.1016\/S0378-1119(96)00589-6"},{"key":"R33","doi-asserted-by":"crossref","unstructured":"O'Donnell LC, Druhan LJ, and Avalos BR.Molecular characterization and expression analysis of leucine-rich \u03b12-glycoprotein, a novel marker of granulocytic differentiation.J Leukoc Biol72: 478\u2013485, 2002.","DOI":"10.1189\/jlb.72.3.478"},{"key":"R34","doi-asserted-by":"publisher","DOI":"10.1006\/bbrc.1998.9911"},{"key":"R35","doi-asserted-by":"crossref","unstructured":"Pencharz PBand Durie PR.Pathogenesis of malnutrition in cystic fibrosis and its treatment.Clin Nutr (Phila)19: 387\u2013394, 2000.","DOI":"10.1054\/clnu.1999.0079"},{"key":"R36","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-200003000-00010"},{"key":"R37","doi-asserted-by":"publisher","DOI":"10.1172\/JCI16521"},{"key":"R38","doi-asserted-by":"publisher","DOI":"10.1378\/chest.121.1.48"},{"key":"R39","doi-asserted-by":"publisher","DOI":"10.1126\/science.2475911"},{"key":"R40","doi-asserted-by":"publisher","DOI":"10.1046\/j.1469-1809.2003.00028.x"},{"key":"R41","doi-asserted-by":"publisher","DOI":"10.1016\/S1074-7613(00)80663-9"},{"key":"R42","doi-asserted-by":"publisher","DOI":"10.1136\/adc.82.5.394"},{"key":"R43","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.98.2.502"},{"key":"R44","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.170.4.1789"},{"key":"R45","doi-asserted-by":"publisher","DOI":"10.1023\/A:1023068116934"},{"key":"R46","doi-asserted-by":"publisher","DOI":"10.1084\/jem.193.4.471"},{"key":"R47","doi-asserted-by":"crossref","unstructured":"Tabary O, Escotte S, Couetil JP, Hubert D, Dusser D, Puchelle E, and Jacquot J.Relationship between I\u03baB\u03b1 deficiency, NF\u03baB activity and interleukin-8 production in CF human airway epithelial cells.Pfl\u00fcgers Arch443,Suppl1: S40\u2013S44, 2001.","DOI":"10.1007\/s004240100642"},{"key":"R48","doi-asserted-by":"publisher","DOI":"10.1177\/002215549804601206"},{"key":"R49","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.00417.2002"},{"key":"R50","doi-asserted-by":"publisher","DOI":"10.1152\/ajpgi.2000.278.3.G343"},{"key":"R51","doi-asserted-by":"publisher","DOI":"10.1016\/S0378-1119(99)00099-2"},{"key":"R52","doi-asserted-by":"publisher","DOI":"10.1128\/IAI.68.4.2142-2147.2000"},{"key":"R53","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-065X.2001.790107.x"}],"container-title":["American Journal of Physiology-Gastrointestinal and Liver Physiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.physiology.org\/doi\/pdf\/10.1152\/ajpgi.00473.2003","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,28]],"date-time":"2023-04-28T15:51:27Z","timestamp":1682697087000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.physiology.org\/doi\/10.1152\/ajpgi.00473.2003"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,6]]},"references-count":53,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2004,6]]}},"alternative-id":["10.1152\/ajpgi.00473.2003"],"URL":"https:\/\/doi.org\/10.1152\/ajpgi.00473.2003","relation":{},"ISSN":["0193-1857","1522-1547"],"issn-type":[{"value":"0193-1857","type":"print"},{"value":"1522-1547","type":"electronic"}],"subject":[],"published":{"date-parts":[[2004,6]]}}}