Pulmonary dysfunction and impaired granulocyte homeostasis result in poor survival of Jam-C-deficient mice

J Pathol. 2007 Jun;212(2):198-208. doi: 10.1002/path.2163.

Abstract

Jam-C(-/-) mice exhibit growth retardation and multilobular pneumonia concomitant with poor survival of the mice under conventional housing conditions. The deficient mice present a mega-oesophagus and have altered airway responsiveness. In addition, the number of circulating granulocytes is increased in Jam-C(-/-) mice as compared to control animals. These phenotypes probably reflect the different functions of JAM-C expressed by endothelial and mesenchymal cells. Indeed, the deregulation in the number of circulating granulocytes is caused by the lack of JAM-C expression on endothelial cells since rescuing endothelial expression of the protein in the Jam-C(-/-) mice is sufficient to restore homeostasis. More importantly, the rescue of vascular JAM-C expression is accompanied by better survival of deficient mice, suggesting that endothelial expression of JAM-C is mandatory for animal survival from opportunistic infections and fatal pneumonia.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Bronchi / immunology
  • Cell Adhesion Molecules / analysis
  • Cell Adhesion Molecules / deficiency*
  • Cell Adhesion Molecules / immunology
  • Disease Susceptibility / immunology
  • Endothelial Cells / immunology
  • Esophageal Achalasia / immunology
  • Esophageal Achalasia / physiopathology
  • Esophagus / immunology
  • Esophagus / physiopathology
  • Granulocytes / immunology*
  • Homeostasis / immunology*
  • Immunoglobulins / analysis
  • Immunoglobulins / deficiency*
  • Immunoglobulins / immunology
  • Immunohistochemistry / methods
  • Leukocyte Count
  • Lung / immunology*
  • Lung / physiopathology
  • Membrane Proteins / analysis
  • Membrane Proteins / deficiency*
  • Membrane Proteins / immunology
  • Mice
  • Mice, Mutant Strains
  • Muscle, Smooth / immunology
  • Neutrophils / immunology
  • Peritonitis / immunology
  • Peritonitis / physiopathology
  • Pneumonia / immunology
  • Receptors, CXCR4 / analysis

Substances

  • CXCR4 protein, mouse
  • Cell Adhesion Molecules
  • Immunoglobulins
  • Jam3 protein, mouse
  • Membrane Proteins
  • Receptors, CXCR4