Targeted disruption of pyrin, the FMF protein, causes heightened sensitivity to endotoxin and a defect in macrophage apoptosis.

@article{Chae2003TargetedDO,
  title={Targeted disruption of pyrin, the FMF protein, causes heightened sensitivity to endotoxin and a defect in macrophage apoptosis.},
  author={Jae Jin Chae and Hirsh D. Komarow and Jun Cheng and Geryl Wood and Nina Raben and Pu Liu and Daniel L. Kastner},
  journal={Molecular cell},
  year={2003},
  volume={11 3},
  pages={591-604}
}
Familial Mediterranean fever (FMF) is an inherited disorder characterized by recurrent episodes of fever and inflammation. Most patients with FMF carry missense mutations in the C-terminal half of the pyrin protein. To study the physiologic role of pyrin, we generated mice expressing a truncated pyrin molecule that, similar to FMF patients, retains the full PYRIN domain. Bacterial lipopolysaccharide (LPS) induces accentuated body temperatures and increased lethality in homozygous mutant mice… CONTINUE READING
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