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Editorial
. 2022 Jan 13:12:831044.
doi: 10.3389/fimmu.2021.831044. eCollection 2021.

Editorial: Function and Dysfunction of Complement Factor H

Mihály Józsi  1 , Paul Nigel Barlow  2 , Seppo Meri  3
Affiliations
Editorial

Editorial: Function and Dysfunction of Complement Factor H

Mihály Józsi et al. Front Immunol. .
No abstract available

Keywords: alternative pathway (AP); complement; factor H (FH); factor H-related protein (FHR); innate immunity.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
The FH family and C3b amplification: (A) FHL-1 and FH contain seven and 20 CCPs (SCRs) respectively. Functional sites on FH are indicated. (B) The CCPs of the FHRs are colour-coded to highlight similarity with CCPs in FH. Dimerisation-mediating CCPs 1-2, are unique to FHRs-1, 2 and 5. Of potential heterodimers, only FHR-2/1 is detectable. (C) In a positive-feedback cycle factor B (FB) binds nascent C3b (that can covalently attach to virtually any surface) whereupon factor D (FD) cleaves FB to yield C3b.Bb, a “C3 convertase”, that cleaves C3 into C3a and C3b. C3b also enters other complement pathways, promoting C5a release and cytolysis. FH destabilises C3b.Bb, competes with FB for binding to C3b, and is a cofactor for factor I (FI) that cleaves C3b to the CR3-ligand iC3b. Some FHRs likely antagonize FH.
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