| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH |
|
|
|
|||||||
|
|||||||||
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
1 Department of Anatomy and Cell Biology, University of Kansas School of Medicine, Kansas City, KS, USA
* To whom correspondence should be addressed. E-mail: rdelisle{at}kumc.edu.
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 2-fold or greater
increase in expression and 98 genes were downregulated 2-fold 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 P450 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 
2 glycoprotein (21-fold),
resistin-like molecule 
(49-fold), and Muclin (2.5-fold); and was significantly decreased for
cytochrome P450 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.
This article has been cited by other articles:
|
|
 |
|
  R. C. De Lisle, W. Xu, B. A. Roe, and D. Ziemer Effects of Muclin (Dmbt1) deficiency on the gastrointestinal system Am J Physiol Gastrointest Liver Physiol, March 1, 2008; 294(3): G717 - G727. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. C. De Lisle, E. Roach, and K. Jansson Effects of laxative and N-acetylcysteine on mucus accumulation, bacterial load, transit, and inflammation in the cystic fibrosis mouse small intestine Am J Physiol Gastrointest Liver Physiol, September 1, 2007; 293(3): G577 - G584. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Wilschanski and P. R Durie Patterns of GI disease in adulthood associated with mutations in the CFTR gene Gut, August 1, 2007; 56(8): 1153 - 1163. [Full Text] [PDF]
|
|
|
|
|
|
J. C. Canale-Zambrano, M. C. Poffenberger, S. M. Cory, D. G. Humes, and C. K. Haston Intestinal phenotype of variable-weight cystic fibrosis knockout mice Am J Physiol Gastrointest Liver Physiol, July 1, 2007; 293(1): G222 - G229. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. C. De Lisle Altered transit and bacterial overgrowth in the cystic fibrosis mouse small intestine Am J Physiol Gastrointest Liver Physiol, July 1, 2007; 293(1): G104 - G111. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 F. Jouret, A. Bernard, C. Hermans, G. Dom, S. Terryn, T. Leal, P. Lebecque, J.-J. Cassiman, B. J. Scholte, H. R. de Jonge, et al. Cystic Fibrosis Is Associated with a Defect in Apical Receptor-Mediated Endocytosis in Mouse and Human Kidney J. Am. Soc. Nephrol., March 1, 2007; 18(3): 707 - 718. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 T. E. Machen Innate immune response in CF airway epithelia: hyperinflammatory? Am J Physiol Cell Physiol, August 1, 2006; 291(2): C218 - C230. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. S. Stalvey, C. Muller, D. A. Schatz, C. H. Wasserfall, M. L. Campbell-Thompson, D. W. Theriaque, T. R. Flotte, and M. A. Atkinson Cystic Fibrosis Transmembrane Conductance Regulator Deficiency Exacerbates Islet Cell Dysfunction After {beta}-Cell Injury. Diabetes, July 1, 2006; 55(7): 1939 - 1945. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. Guilbault, J. P. Novak, P. Martin, M.-L. Boghdady, Z. Saeed, M.-C. Guiot, T. J. Hudson, and D. Radzioch Distinct pattern of lung gene expression in the Cftr-KO mice developing spontaneous lung disease compared with their littermate controls Physiol Genomics, April 13, 2006; 25(2): 179 - 193. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 O. Norkina, T. G. Burnett, and R. C. De Lisle Bacterial Overgrowth in the Cystic Fibrosis Transmembrane Conductance Regulator Null Mouse Small Intestine Infect. Immun., October 1, 2004; 72(10): 6040 - 6049. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH |
| Visit Other APS Journals Online |
