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.
Factor H family proteins: on complement, microbes and human diseases
- Views Icon Views
- Share Icon Share
P. F. Zipfel, C. Skerka, J. Hellwage, S. T. Jokiranta, S. Meri, V. Brade, P. Kraiczy, M. Noris, G. Remuzzi; Factor H family proteins: on complement, microbes and human diseases. Biochem Soc Trans 1 November 2002; 30 (6): 971–978. doi: https://doi.org/10.1042/bst0300971
Download citation file: