Distinct pattern of lung gene expression in the Cftr-KO mice developing spontaneous lung disease compared with their littermate controls

Physiol Genomics. 2006 Apr 13;25(2):179-93. doi: 10.1152/physiolgenomics.00206.2005. Epub 2006 Jan 17.


Cystic fibrosis (CF) is caused by a defect in the CF transmembrane conductance regulator (CFTR) protein that functions as a chloride channel. Dysfunction of the CFTR protein results in salty sweat, pancreatic insufficiency, intestinal obstruction, male infertility, and severe pulmonary disease. Most of the morbidity and mortality of CF patients results from pulmonary complications. Differences in susceptibility to bacterial infection and variable degree of CF lung disease among CF patients remain unexplained. Many phenotypic expressions of the disease do not directly correlate with the type of mutation in the Cftr gene. Using a unique CF mouse model that mimics aspects of human CF lung disease, we analyzed the differential gene expression pattern between the normal lungs of wild-type mice (WT) and the affected lungs of CFTR knockout mice (KO). Using microarray analysis followed by quantitation of candidate gene mRNA and protein expression, we identified many interesting genes involved in the development of CF lung disease in mice. These findings point to distinct mechanisms of gene expression regulation between mice with CF and control mice.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cystic Fibrosis / genetics
  • Cystic Fibrosis / metabolism*
  • Cystic Fibrosis / pathology
  • Disease Models, Animal
  • Female
  • Gene Expression Regulation*
  • Immunoglobulin G / genetics
  • Immunoglobulin G / metabolism
  • Lung / metabolism*
  • Lung / pathology
  • Male
  • Matrix Metalloproteinase 9 / genetics
  • Matrix Metalloproteinase 9 / metabolism
  • Mice
  • Mice, Inbred CFTR
  • Oligonucleotide Array Sequence Analysis / methods
  • Pneumonia, Bacterial / genetics
  • Pneumonia, Bacterial / metabolism*
  • Pneumonia, Bacterial / pathology
  • Pseudomonas Infections / genetics
  • Pseudomonas Infections / metabolism*
  • Pseudomonas Infections / pathology
  • RNA, Messenger / metabolism
  • Reproducibility of Results
  • Secretory Leukocyte Peptidase Inhibitor / genetics
  • Secretory Leukocyte Peptidase Inhibitor / metabolism


  • Immunoglobulin G
  • RNA, Messenger
  • Secretory Leukocyte Peptidase Inhibitor
  • Slpi protein, mouse
  • Matrix Metalloproteinase 9