Cfp

Summary

Gene Symbol: Cfp
Description: complement factor properdin
Alias: BCFG, Pfc, properdin, complement factor P, properdin factor, complement
Species: mouse
Products:     Cfp

Top Publications

  1. Ivanovska N, Dimitrova P, Luckett J, El Rachkidy Lonnen R, Schwaeble W, Stover C. Properdin deficiency in murine models of nonseptic shock. J Immunol. 2008;180:6962-9 pubmed
    Hereditary properdin deficiency is linked to susceptibility to meningococcal disease (Neisseria meningitidis serotypes Y and W-135) with high mortality...
  2. Dimitrova P, Ivanovska N, Schwaeble W, Gyurkovska V, Stover C. The role of properdin in murine zymosan-induced arthritis. Mol Immunol. 2010;47:1458-66 pubmed publisher
    Using properdin-deficient and wild-type mice, we have investigated the role of properdin in development and progression of zymosan-induced arthritis...
  3. Stover C, Luckett J, Echtenacher B, Dupont A, Figgitt S, Brown J, et al. Properdin plays a protective role in polymicrobial septic peritonitis. J Immunol. 2008;180:3313-8 pubmed
    b>Properdin is a positive regulator of complement activation so far known to be instrumental in the survival of infections with certain serotypes of Neisseria meningitidis...
  4. Ali Y, Hayat A, Saeed B, Haleem K, Alshamrani S, Kenawy H, et al. Low-dose recombinant properdin provides substantial protection against Streptococcus pneumoniae and Neisseria meningitidis infection. Proc Natl Acad Sci U S A. 2014;111:5301-6 pubmed publisher
    ..Here we report the successful therapeutic application of a complement component, a recombinant form of properdin (Pn), with significantly higher activity than native properdin, which promotes complement activation via the ..
  5. Gormally E, Boyd Y. Faint lined (Fnl): a novel X-linked coat mutant in the mouse. Genet Res. 1998;72:211-6 pubmed
    ..Further genetic resolution of co-segregating markers flanking Fnl established that Fnl lies in a 7.6 +/- 2.6 cM interval between DXMit50 and DXMit82. ..
  6. Lesher A, Zhou L, Kimura Y, Sato S, Gullipalli D, Herbert A, et al. Combination of factor H mutation and properdin deficiency causes severe C3 glomerulonephritis. J Am Soc Nephrol. 2013;24:53-65 pubmed publisher
    Factor H (fH) and properdin both modulate complement; however, fH inhibits activation, and properdin promotes activation of the alternative pathway of complement...
  7. Laval S, Chen Z, Boyd Y. The properdin structural locus (Pfc) lies close to the locus for tissue inhibitor of metallothionine proteases (Timp) on the mouse X chromosome. Genomics. 1991;10:1030-4 pubmed
    We have confirmed the assignment of the structural locus of the complement factor properdin (Pfc) to the mouse X chromosome and mapped it between monoamine oxidase-A (Mao-a) and hypoxanthine phosphoribosyltransferase (Hprt) using a Mus ..
  8. Freeley S, Popat R, Parmar K, Kolev M, Hunt B, Stover C, et al. Experimentally-induced anti-myeloperoxidase vasculitis does not require properdin, MASP-2 or bone marrow-derived C5. J Pathol. 2016;240:61-71 pubmed publisher
    ..We showed that neither MASP-2- nor properdin-deficient mice were protected, suggesting that alternative pathway activation does not require properdin or the ..
  9. Dupont A, Mohamed F, Salehen N, Glenn S, Francescut L, Adib R, et al. Septicaemia models using Streptococcus pneumoniae and Listeria monocytogenes: understanding the role of complement properdin. Med Microbiol Immunol. 2014;203:257-71 pubmed publisher
    ..and Listeria monocytogenes, pathogens which can cause severe infectious disease in human, were used to infect properdin-deficient and wildtype mice...

More Information

Publications26

  1. Miwa T, Sato S, Gullipalli D, Nangaku M, Song W. Blocking properdin, the alternative pathway, and anaphylatoxin receptors ameliorates renal ischemia-reperfusion injury in decay-accelerating factor and CD59 double-knockout mice. J Immunol. 2013;190:3552-9 pubmed publisher
    ..mice with mice deficient in various complement components or receptors including C3, C4, factor B (fB), factor properdin (fP), mannose-binding lectin, C3aR, C5aR, or Ig and assessed renal IRI in the resulting mutant strains...
  2. Kimura Y, Zhou L, Miwa T, Song W. Genetic and therapeutic targeting of properdin in mice prevents complement-mediated tissue injury. J Clin Invest. 2010;120:3545-54 pubmed
    ..Dysfunction of AP regulatory proteins has been linked to several human inflammatory disorders. Properdin is a positive regulator of AP complement activation that has been shown to extend the half-life of cell surfaceâ€..
  3. Evans E, Burtenshaw M, Laval S, Goundis D, Reid K, Boyd Y. Localization of the properdin factor complement locus Pfc to band A3 on the mouse X chromosome. Genet Res. 1990;56:153-5 pubmed
    The locus for properdin (properdin factor complement, Pfc), a plasma glycoprotein, has been mapped to band A3 of the mouse X chromosome by in situ hybridization to metaphase spreads containing an X;2 Robertsonian translocation...
  4. Lyzogubov V, Bora P, Wu X, Horn L, de Roque R, Rudolf X, et al. The Complement Regulatory Protein CD46 Deficient Mouse Spontaneously Develops Dry-Type Age-Related Macular Degeneration-Like Phenotype. Am J Pathol. 2016;186:2088-2104 pubmed publisher
    ..More important, tissue-specific expression of CD46 gives the Cd46(-/-) mouse model of dry AMD a unique advantage over other mouse models using knockout strains. ..
  5. Zhou H, Yan H, Stover C, Fernandez T, Rodriguez de Cordoba S, Song W, et al. Antibody directs properdin-dependent activation of the complement alternative pathway in a mouse model of abdominal aortic aneurysm. Proc Natl Acad Sci U S A. 2012;109:E415-22 pubmed publisher
    ..However, the mechanism by which complement AP is initiated remains undefined. The complement protein properdin, traditionally viewed as a positive regulator of the AP, may also initiate complement activation by binding ..
  6. Kimura Y, Miwa T, Zhou L, Song W. Activator-specific requirement of properdin in the initiation and amplification of the alternative pathway complement. Blood. 2008;111:732-40 pubmed
    b>Properdin is a positive regulator of alternative pathway (AP) complement. The current understanding of properdin function is that it facilitates AP complement activation by stabilizing the C3 convertase C3bBb...
  7. Blair H, Ho M, Monaco A, Fisher S, Craig I, Boyd Y. High-resolution comparative mapping of the proximal region of the mouse X chromosome. Genomics. 1995;28:305-10 pubmed
    ..short arm, the position of evolutionary breakpoints with respect to key loci has been established as DMD-EB-XK-PFC-EB-GATA1-C1CN5-EB-DXS1272E-ALAS2-E B-DXF34-centromere...
  8. Goundis D, Reid K. Properdin, the terminal complement components, thrombospondin and the circumsporozoite protein of malaria parasites contain similar sequence motifs. Nature. 1988;335:82-5 pubmed
    b>Properdin is a plasma glycoprotein which stabilizes the C3bnBb enzyme complex of the alternative pathway of the complement system...
  9. Gauvreau D, Roy C, Tom F, Lu H, Miegueu P, Richard D, et al. A new effector of lipid metabolism: complement factor properdin. Mol Immunol. 2012;51:73-81 pubmed publisher
    ..b>Properdin stabilizes C3 convertase following alternative complement activation...
  10. Ruseva M, Vernon K, Lesher A, Schwaeble W, Ali Y, Botto M, et al. Loss of properdin exacerbates C3 glomerulopathy resulting from factor H deficiency. J Am Soc Nephrol. 2013;24:43-52 pubmed publisher
    Complement factor H (CFH) is a negative regulator of the alternative pathway of complement, and properdin is the sole positive regulator...
  11. Farrar J, Howard M, Fuller Farrar J, Paul W. Biochemical and physicochemical characterization of mouse B cell growth factor: a lymphokine distinct from interleukin 2. J Immunol. 1983;131:1838-42 pubmed
    ..The biologic activity of IL 2 is sensitive to trypsin inactivation as is BCGF activity. Thus, BCGF and IL 2 appear to be biologically and biochemically distinct glycoproteins. ..
  12. Jain U, Midgen C, Woodruff T, Schwaeble W, Stover C, Stadnyk A. Properdin deficiency protects from 5-fluorouracil-induced small intestinal mucositis in a complement activation-independent, interleukin-10-dependent mechanism. Clin Exp Immunol. 2017;188:36-44 pubmed publisher
    ..We have previously shown an effect of properdin, a positive regulator of complement activation, in models of colitis...
  13. Gulati S, Agarwal S, Vasudhev S, Rice P, Ram S. Properdin is critical for antibody-dependent bactericidal activity against Neisseria gonorrhoeae that recruit C4b-binding protein. J Immunol. 2012;188:3416-25 pubmed publisher
    ..The role of AP recruitment and, in particular, properdin in assisting killing of gonococci by specific Abs is the subject of this study...
  14. Steiner T, Francescut L, Byrne S, Hughes T, Jayanthi A, Guschina I, et al. Protective role for properdin in progression of experimental murine atherosclerosis. PLoS ONE. 2014;9:e92404 pubmed publisher
    ..The serum glycoprotein properdin is a key positive regulator of the AP but has not been studied in experimental atherosclerosis...
  15. Laval S, Boyd Y. Novel sequences conserved on the human and mouse X chromosomes. Genomics. 1993;15:483-91 pubmed
    ..The mapping of another probe that detects a novel sequence family (DXF34) close to the X chromosome centromere in both species suggests that a block of pericentromeric material is conserved between the X chromosomes of man and mouse. ..
  16. Battinelli E, Boyd Y, Craig I, Breakefield X, Chen Z. Characterization and mapping of the mouse NDP (Norrie disease) locus (Ndp). Mamm Genome. 1996;7:93-7 pubmed
    ..Pedigree analysis of an interspecific mouse backcross localizes the mouse NDP gene close to Maoa in the conserved segment, which runs from CYBB to PFC in both human and mouse.
  17. Wang Y, Miwa T, Ducka Kokalari B, Redai I, Sato S, Gullipalli D, et al. Properdin Contributes to Allergic Airway Inflammation through Local C3a Generation. J Immunol. 2015;195:1171-81 pubmed publisher
    ..In this study, we investigated the role of properdin (P), a positive alternative pathway complement regulator, in allergen-induced airway inflammation...