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Cfp  -  complement factor properdin

Mus musculus

Synonyms: BCFG, Complement factor P, Pfc, Properdin
 
 
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Disease relevance of Cfp

  • In an in vivo model of polymicrobial septic peritonitis induced by sublethal cecal ligation and puncture, properdin-deficient mice appear immunocompromised, because they are significantly impaired in their survival compared with wild-type littermates [1].
  • These results reveal a novel role of properdin in AP complement initiation and have implications for understanding the selective predisposition of properdin-deficient patients to meningococcal infection [2].
 

High impact information on Cfp

 

Associations of Cfp with chemical compounds

  • Properdin-deficient mice showed significantly higher mortality in LPS shock, elevated TNF-alpha, and, inversely, reduced IL-10 production by peritoneal macrophages as well as lower plasma C5a levels compared with wild-type littermates [4].

References

  1. Properdin plays a protective role in polymicrobial septic peritonitis. Stover, C.M., Luckett, J.C., Echtenacher, B., Dupont, A., Figgitt, S.E., Brown, J., Männel, D.N., Schwaeble, W.J. J. Immunol. (2008) [Pubmed]
  2. Activator-specific requirement of properdin in the initiation and amplification of the alternative pathway complement. Kimura, Y., Miwa, T., Zhou, L., Song, W.C. Blood (2008) [Pubmed]
  3. The properdin structural locus (Pfc) lies close to the locus for tissue inhibitor of metallothionine proteases (Timp) on the mouse X chromosome. Laval, S.H., Chen, Z.Y., Boyd, Y. Genomics (1991) [Pubmed]
  4. Properdin deficiency in murine models of nonseptic shock. Ivanovska, N.D., Dimitrova, P.A., Luckett, J.C., El-Rachkidy Lonnen, R., Schwaeble, W.J., Stover, C.M. J. Immunol. (2008) [Pubmed]
 
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