Encyclopedia of Signaling Molecules

2018 Edition
| Editors: Sangdun Choi

Complement Factor H (CFH)

  • Eduardo Lamas-Basulto
  • Anne M. Dersch
  • Claudio Cortes
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-67199-4_101784

Synonyms

Historical Background

The complement system, the major noncellular component of the innate immune system, is composed of three different pathways: the classical (CP), the lectin (LP), and the alternative pathway (AP). These pathways participate in several processes, including tagging pathogens for phagocytosis, directing destruction of microorganisms, promoting inflammation, contributing to immune complex regulation, and linking the innate and adaptive immune systems. The complement system has two fundamental properties: discriminates self (e.g., host cells) from nonself (e.g., pathogens) and regulates itself to prevent unwanted damage. These properties, common to all arms of the immune system, are required for proper protection and...

This is a preview of subscription content, log in to check access.

References

  1. Abdul-Aziz M, Tsolaki AG, Kouser L, Carroll MV, Al-Ahdal MN, Sim RB, Kishore U. Complement factor H interferes with Mycobacterium bovis BCG entry into macrophages and modulates the pro-inflammatory cytokine response. Immunobiology. 2016;221:944–52.PubMedCrossRefGoogle Scholar
  2. Amdahl H, Jarva H, Haanperä M, Mertsola J, He Q, Jokiranta TS, Meri S. Interactions between Bordetella pertussis and the complement inhibitor factor H. Mol Immunol. 2011;48:697–705.PubMedCrossRefGoogle Scholar
  3. Appelboom G, Piazza M, Hwang BY, Bruce S, Smith S, Bratt A, Bagiella E, Badjatia N, Mayer S, Sander CE. Complement factor H Y402H polymorphism is associated with an increased risk of mortality after intracerebral hemorrhage. J Clin Neurosci. 2011;18:1439–43.PubMedCrossRefGoogle Scholar
  4. Bernhard S, Fleury C, Su YC, Zipfel PF, Koske I, Nordström T, Riesbeck K. Outer membrane protein OlpA contributes to Moraxella catarrhalis serum resistance via interaction with factor H and the alternative pathway. J Infect Dis. 2014;210:1306–10.PubMedCrossRefGoogle Scholar
  5. Blatt AZ, Pathan S, Ferreira VP. Properdin: a tightly regulated critical inflammatory modulator. Immunol Rev. 2016;274:172–90.PubMedPubMedCentralCrossRefGoogle Scholar
  6. Blaum BS, Hannan JP, Herbert AP, Kavanagh D, Uhrín D, Stehle T. Structural basis for sialic acid-mediated self-recognition by complement factor H. Nat Chem Biol. 2015;11:77–82.PubMedCrossRefGoogle Scholar
  7. Castiblanco-Valencia MM, Fraga TR, Silva LB, Monaris D, Abreu PA, Strobel S, Józsi M, Isaac L, Barbosa AS. Leptospiral immunoglobulin-like proteins interact with human complement regulators factor H, FHL-1, FHR-1, and C4BP. J Infect Dis. 2012;205:995–04.PubMedCrossRefGoogle Scholar
  8. Clark SJ, Ridge LA, Herbert AP, Hakobyan S, Mulloy B, Lennon R, Würzner R, Morgan BP, Uhrín D, Bishop PN, Day AJ. Tissue-specific host recognition by complement factor H is mediated by differential activities of its glycosaminoglycan-binding regions. J Immunol. 2013;190:2049–57.PubMedPubMedCentralCrossRefGoogle Scholar
  9. Cortes C, Ohtola JA, Saggu G, Ferreira VP. Local release of properdin in the cellular microenvironment: role in pattern recognition and amplification of the alternative pathway of complement. Front Immunol. 2013;3:412.PubMedPubMedCentralCrossRefGoogle Scholar
  10. de Cordoba SR, de Jorge EG. Translational mini-review series on complement factor H: genetics and disease associations of human complement factor H. Clin Exp Immunol. 2008;151:1–13.PubMedPubMedCentralCrossRefGoogle Scholar
  11. Du H, Xiao X, Stiles T, Douglas C, Ho D, Shaw PX. Novel Mechanistic interplay between products of oxidative stress and components of the complement system in AMD pathogenesis. Open J Ophthalmol. 2016;6:43–50.PubMedPubMedCentralCrossRefGoogle Scholar
  12. Fearon DT. Regulation by membrane sialic acid of BIH-dependent decay-dissociation of amplification C3 convertase of the alternative complement pathway. Proc Natl Acad Sci U S A. 1978;75:1971–5.PubMedPubMedCentralCrossRefGoogle Scholar
  13. Feng S, Liang X, Cruz MA, Vu H, Zhou Z, Pemmaraju N, Dong JF, Kroll MH, Afshar-Kharghan V. The interaction between factor H and Von Willebrand factor. PLoS One. 2013;8:e73715.PubMedPubMedCentralCrossRefGoogle Scholar
  14. Ferreira VP, Herbert AP, Hocking HG, Barlow PN, Pangburn MK. Critical role of the C-terminal domains of factor H in regulating complement activation at cell surfaces. J Immunol. 2006;177:6308–16.PubMedCrossRefGoogle Scholar
  15. Ferreira VP, Pangburn MK. Factor H mediated cell surface protection from complement is critical for the survival of PNH erythrocytes. Blood. 2007;110:2190–2.PubMedPubMedCentralCrossRefGoogle Scholar
  16. Ferreira VP, Pangburn MK, Cortés C. Complement control protein factor H: the good, the bad, and the inadequate. Mol Immunol. 2010;47:2187–97.PubMedPubMedCentralCrossRefGoogle Scholar
  17. Li W, Wen L, Li C, Chen R, Ye Z, Zhao J, Pan J. Contribution of the outer membrane protein OmpW in Escherichia coli to complement resistance from binding to factor. Microb Pathog. 2016;98:57–62.PubMedCrossRefGoogle Scholar
  18. Haapasalo K, van Kessel K, Nissilä E, Metso J, Johansson T, Miettinen S, Varjosalo M, Kirveskari J, Kuusela P, Chroni A, Jauhiainen M, van Strijp J, Jokiranta TS. Complement factor H binds to human serum Apolipoprotein E and mediates complement regulation on high density lipoprotein particles. J Biol Chem. 2015;290:28977–87.PubMedPubMedCentralCrossRefGoogle Scholar
  19. Hamad OA, Nilsson PH, Lasaosa M, Ricklin D, Lambris JD, Nilsson B, Ekdahl KN. Contribution of chondroitin sulfate A to the binding of complement proteins to activated platelets. PLoS One. 2010;5:e12889.PubMedPubMedCentralCrossRefGoogle Scholar
  20. Heurich M, Preston RJ, O'Donnell VB, Morgan BP, Collins PW. Thrombomodulin enhances complement regulation through strong affinity interactions with factor H and C3b-factor H complex. Thromb Res. 2016;145:84–92.PubMedCrossRefGoogle Scholar
  21. Ho DK, Jarva H, Meri S. Human complement factor H binds to outer membrane protein Rck of Salmonella. J Immunol. 2010;185:1763–9.PubMedCrossRefGoogle Scholar
  22. Ho DK, Riva R, Skurnik M, Meri S. The Yersinia pseudotuberculosis outer membrane protein Ail recruits the human complement regulatory protein factor H. J Immunol. 2012;189:3593–9.PubMedCrossRefGoogle Scholar
  23. Holers VM. Complement and its receptors: new insights into human disease. Annu Rev Immunol. 2014;32:433–59.PubMedCrossRefGoogle Scholar
  24. Józsi M, Tortajada A, Uzonyi B, Goicoechea de Jorge E, Rodríguez de Córdoba S. Factor H-related proteins determine complement-activating surfaces. Trends Immunol. 2015;36:374–84.PubMedCrossRefGoogle Scholar
  25. Kennedy AT, Schmidt CQ, Thompson JK, Weiss GE, Taechalertpaisarn T, Gilson PR, Barlow PN, Crabb BS, Cowman AF, Tham WH. Recruitment of factor H as a novel complement evasion strategy for blood-stage plasmodium falciparum infection. J Immunol. 2016;196:1239–48.PubMedCrossRefGoogle Scholar
  26. Kondo N, Honda S, Kuno S, Negi A. Coding variant I62V in the complement factor H gene is strongly associated with polypoidal choroidal vasculopathy. Ophthalmology. 2009;116:304–10.PubMedCrossRefGoogle Scholar
  27. Maller J, George S, Purcell S, Fagerness J, Altshuler D, Daly MJ, Seddon JM. Common variation in three genes, including a noncoding variant in CFH, strongly influences risk of age-related macular degeneration. Nat Genet. 2006;38:1055–9.PubMedCrossRefGoogle Scholar
  28. Nester CM, Barbour T, de Cordoba SR, Dragon-Durey MA, Fremeaux-Bacchi V, Goodship TH, Kavanagh D, Noris M, Pickering M, Sanchez-Corral P, Skerka C, Zipfel P, Smith RJ. Atypical aHUS: state of the art. Mol Immunol. 2015;67:31–42.PubMedCrossRefGoogle Scholar
  29. Nishio N, Teranishi M, Uchida Y, Sugiura S, Ando F, Shimokata H, Sone M, Otake H, Kato K, Yoshida T, Tagaya M, Hibi T, Nakashima T. Contribution of complement factor H Y402H polymorphism to sudden sensorineural hearing loss risk and possible interaction with diabetes. Gene. 2012;499:226–30.PubMedCrossRefGoogle Scholar
  30. Pangburn MK, Ferreira VP, Cortes C. Discrimination between host and pathogens by the complement system. Vaccine. 2008;26:I15–21.PubMedPubMedCentralCrossRefGoogle Scholar
  31. Pangburn MK, Rawal N, Cortes C, Alam MN, Ferreira VP, Atkinson MA. Polyanion-induced self-association of complement factor H. J Immunol. 2009;182:1061–8.PubMedPubMedCentralCrossRefGoogle Scholar
  32. Pangburn MK, Schreiber RD, Muller-Eberhard HJ. Human complement C3b inactivator: Isolation, characterization, and demonstration of an absolute requirement for the serum protein BIH for cleavage of C3b and C4b in solution. J Exp Med. 1977;146:257–70.PubMedCrossRefPubMedCentralGoogle Scholar
  33. Perkins SJ, Fung KW, Khan S. Molecular interactions between complement factor H and its Heparin and Heparan Sulfate Ligands. Front Immunol. 2014;5:126.PubMedPubMedCentralCrossRefGoogle Scholar
  34. Pian Y, Gan S, Wang S, Guo J, Wang P, Zheng Y, Cai X, Jiang Y, Yuan Y. Fhb, a novel factor H-binding surface protein, contributes to the antiphagocytic ability and virulence of Streptococcus suis. Infect Immun. 2012;80:2402–13.PubMedPubMedCentralCrossRefGoogle Scholar
  35. Pickering MC, de Jorge EG, Martinez-Barricarte R, Recalde S, Garcia-Layana A, Rose KL, Moss J, Walport MJ, Cook HT, de Córdoba SR, Botto M. Spontaneous hemolytic uremic syndrome triggered by complement factor H lacking surface recognition domains. J Exp Med. 2007;204:1249–56.PubMedPubMedCentralCrossRefGoogle Scholar
  36. Raychaudhuri S, Iartchouk O, Chin K, Tan PL, Tai AK, Ripke S, Gowrisankar S, Vemuri S, Montgomery K, Yu Y, Reynolds R, Zack DJ, Campochiaro B, Campochiaro P, Katsanis N, Daly MJ, Seddon JM. A rare penetrant mutation in CFH confers high risk of age-related macular degeneration. Nat Genet. 2011;43:1232–6.PubMedPubMedCentralCrossRefGoogle Scholar
  37. Renner B, Ferreira VP, Cortes C, Goldberg R, Ljubanovic D, Pangburn MK, Pickering MC, Tomlinson S, Holland-Neidermyer A, Strassheim D, Holers VM, Thurman JM. Binding of factor H to tubular epithelial cells limits interstitial complement activation in ischemic injury. Kidney Int. 2011;80:165–73.PubMedPubMedCentralCrossRefGoogle Scholar
  38. Riva R, Korhonen TK, Meri S. The outer membrane protease PgtE of Salmonella enterica interferes with the alternative complement pathway by cleaving factors B and H. Front Microbiol. 2015;6:63.PubMedPubMedCentralCrossRefGoogle Scholar
  39. Rosa TF, Flammersfeld A, Ngwa CJ, Kiesow M, Fischer R, Zipfel PF, Skerka C, Pradel G. The Plasmodium falciparum blood stages acquire factor H family proteins to evade destruction by human complement. Cell Microbiol. 2016;18:573–90.PubMedCrossRefGoogle Scholar
  40. Sahagún-Ruiz A, Granados Martinez AP, Breda LC, Fraga TR, Castiblanco Valencia MM, Barbosa AS, Isaac L. Pasteurella pneumotropica evades the human complement system by acquisition of the complement regulators factor H and C4BP. PLoS One. 2014;9:e111194.PubMedPubMedCentralCrossRefGoogle Scholar
  41. Schmidt CQ, Lambris JD, Ricklin D. Protection of host cells by complement regulators. Immunol Rev. 2016;274(1):152–71.PubMedPubMedCentralCrossRefGoogle Scholar
  42. Schreiber RD, Pangburn MK, Lesavre P, Muller-Eberhard HJ. Initiation of the alternative pathway of complement: recognition of activators by bound C3b and assembly of the entire pathway from six isolated proteins. Proc Natl Acad Sci U S A. 1978;75:3948–52.PubMedPubMedCentralCrossRefGoogle Scholar
  43. Schwaeble W, Zwirner J, Schulz TF, Linke RP, Dierich MP, Weiss EH. Human complement factor H: expression of an additional truncated gene product of 43 kDa in human liver. Eur J Immunol. 1987;17:1485–9.PubMedCrossRefGoogle Scholar
  44. Sharp JA, Echague CG, Hair PS, Ward MD, Nyalwidhe JO, Geoghegan JA, Foster TJ, Cunnion KM. Staphylococcus aureus surface protein SdrE binds complement regulator factor H as an immune evasion tactic. PLoS One. 2012;7(5):e38407.PubMedPubMedCentralCrossRefGoogle Scholar
  45. Shaw PX, Zhang L, Zhang M, Du H, Zhao L, Lee C, Grob S, Lim SL, Hughes G, Lee J, Bedell M, Nelson MH, Lu F, Krupa M, Luo J, Ouyang H, Tu Z, Su Z, Zhu J, Wei X, Feng Z, Duan Y, Yang Z, Ferreyra H, Bartsch DU, Kozak I, Zhang L, Lin F, Sun H, Feng H, Zhang K. Complement factor H genotypes impact risk of age-related macular degeneration by interaction with oxidized phospholipids. Proc Natl Acad Sci U S A. 2012;109:13757–62.PubMedPubMedCentralCrossRefGoogle Scholar
  46. Simon N, Lasonder E, Scheuermayer M, Kuehn A, Tews S, Fischer R, Zipfel PF, Skerka C, Pradel G. Malaria parasites co-opt human factor H to prevent complement-mediated lysis in the mosquito midgut. Cell Host Microbe. 2013;13:29–41.PubMedCrossRefGoogle Scholar
  47. Wang Y, Jenkins SA, Gu C, Shree A, Martinez-Moczygemba M, Herold J, Botto M, Wetsel RA, Xu Y. Bacillus anthracis spore surface protein BclA mediates complement factor H binding to spores and promotes spore persistence. PLoS Pathog. 2016;12:e1005678.PubMedPubMedCentralCrossRefGoogle Scholar
  48. Weiler JM, Daha MR, Austen KF, Fearon DT. Control of the amplification convertase of complement by the plasma protein BIH. Proc Natl Acad Sci U S A. 1976;73:3268–72.PubMedPubMedCentralCrossRefGoogle Scholar
  49. Weismann D, Hartvigsen K, Lauer N, Bennett KL, Scholl HP, Charbel Issa P, Cano M, Brandstätter H, Tsimikas S, Skerka C, Superti-Furga G, Handa JT, Zipfel PF, Witztum JL, Binder CJ. Complement factor H binds malondialdehyde epitopes and protects from oxidative stress. Nature. 2011;478:76–81.PubMedPubMedCentralCrossRefGoogle Scholar
  50. Whaley K, Ruddy S. Modulation of the alternative complement pathway by BIH globulin. J Exp Med. 1976;144:1147–63.PubMedCrossRefGoogle Scholar
  51. Xing C, Sivakumaran TA, Wang JJ, Rochtchina E, Joshi T, Smith W, Mitchell P, Lyengar SK. Complement factor H polymorphisms, renal phenotypes and age-related macular degeneration: the Blue Mountains Eye Study. Genes Immun. 2008;9:231–9.PubMedCrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  • Eduardo Lamas-Basulto
    • 1
  • Anne M. Dersch
    • 1
  • Claudio Cortes
    • 1
  1. 1.Oakland University William Beaumont School of MedicineRochesterUSA