The complement factor H‐related proteins

N Medjeral‐Thomas, MC Pickering - Immunological Reviews, 2016 - Wiley Online Library
The role of the complement factor H‐related (FHR) proteins in homeostasis, pathogen
defense, and autoimmune disease has recently attracted considerable interest. We highlight …

Modulation of the alternative pathway of complement by murine factor H–related proteins

AH Antonioli, J White, F Crawford, B Renner… - The Journal of …, 2018 - journals.aai.org
Factor H (FH) is a key alternative pathway regulator that controls complement activation both
in the fluid phase and on specific cell surfaces, thus allowing the innate immune response to …

Factor H-related proteins 1–5

SR Barnum, PN Barlow - The Complement FactsBook, 2018 - Elsevier
This chapter provides an overview of the state of knowledge of the set of five complement
factor H-related proteins (CFHRs) in humans. A brief description is included of their …

Function and dysfunction of complement factor h

M Józsi, PN Barlow, S Meri - Frontiers in Immunology, 2022 - frontiersin.org
The complement system responds very quickly to danger due to the eternal vigilance of its
alternative pathway (AP). In the AP, an always-on positive-feedback C3b-amplification loop …

[HTML][HTML] The SCR-17 and SCR-18 glycans in human complement Factor H enhance its regulatory function

X Gao, H Iqbal, DQ Yu, J Gor, AR Coker… - Journal of Biological …, 2024 - Elsevier
Human complement factor H (CFH) plays a central role in regulating activated C3b to protect
host cells. CFH contain 20 short complement regulator (SCR) domains and eight N …

The murine factor H-related protein FHR-B promotes complement activation

M Cserhalmi, ÁI Csincsi, Z Mezei, A Kopp… - Frontiers in …, 2017 - frontiersin.org
Factor H-related (FHR) proteins consist of varying number of complement control protein
domains that display various degrees of sequence identity to respective domains of the …

Structure of complement fragment C3b–factor H and implications for host protection by complement regulators

J Wu, YQ Wu, D Ricklin, BJC Janssen, JD Lambris… - Nature …, 2009 - nature.com
Factor H (FH) is an abundant regulator of complement activation and protects host cells from
self-attack by complement. Here we provide insight into the regulatory activity of FH by …

Factor H–related protein 1 (CFHR-1) inhibits complement C5 convertase activity and terminal complex formation

S Heinen, A Hartmann, N Lauer, U Wiehl… - Blood, The Journal …, 2009 - ashpublications.org
Homozygous deletion of a 84-kb genomic fragment in human chromosome 1 that
encompasses the CFHR1 and CFHR3 genes represents a risk factor for hemolytic uremic …

Mapping the complement factor H-related protein 1 (CFHR1): C3b/C3d interactions

JP Hannan, J Laskowski, JM Thurman, GS Hageman… - PloS one, 2016 - journals.plos.org
Complement factor H-related protein 1 (CFHR1) is a complement regulator which has been
reported to regulate complement by blocking C5 convertase activity and interfering with C5b …

Factor H-related protein 4 activates complement by serving as a platform for the assembly of alternative pathway C3 convertase via its interaction with C3b protein

M Hebecker, M Józsi - Journal of biological chemistry, 2012 - ASBMB
Human complement factor H-related protein (CFHR) 4 belongs to the factor H family of
plasma glycoproteins that are composed of short consensus repeat (SCR) domains …