Factor H family proteins: On complement, microbes and human diseases

P. F. Zipfel, C. Skerka, J. Hellwage, S. T. Jokiranta, S. Meri, V. Brade, P. Kraiczy, M. Noris, G. Remuzzi

Research output: Contribution to journalArticlepeer-review

Abstract

At present, the human Factor H protein family represents seven multidomain, multifunctional serum proteins. This group includes the complement and immune regulators Factor H, the Factor H-like protein 1 (FHL-1) and five Factor H-related proteins proteins (FHR-1, -2, -3, -4 and -5). Each is exclusively composed of individually folded protein domains, termed short consensus repeats (SCRs) or complement control modules. Structure-function analyses allowed the localization of the complement regulatory domain of Factor H and FHL-1 in the N-terminal region within SCRs 1-4. In addition, multiple binding sites for C3b, heparin and microbial surface proteins were localized in the N-terminus, within the middle region and also in the C-terminus of Factor H and FHL-1. Recent results show a central role for the C-terminus of Factor H, i.e. SCRs 19-20. These particular domains are conserved in all FHRs identified so far, include contact points for C3b, heparin and microbial surface proteins and represent a 'hot-spot' for gene mutations in patients that suffer from the Factor H-associated form of haemolytic uraemic syndrome.

Original languageEnglish
Pages (from-to)971-978
Number of pages8
JournalBiochemical Society Transactions
Volume30
Issue number6
DOIs
Publication statusPublished - Nov 2002

Keywords

  • Complement regulation
  • Factor H protein family
  • Haemolytic uraemic syndrome
  • Immunevasion
  • Innate immunity
  • Microbial evasion strategy

ASJC Scopus subject areas

  • Biochemistry

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