The complement factor H‐related proteins
SummaryThe role of the complement factor H‐related (FHR) proteins in homeostasis, pathogen defense, and autoimmune disease has recently attracted considerable interest. We highlight the exciting research that has contributed to our understanding of the FHR protein family. Unlike factor H, a potent negative regulator of complement C3 activation, the FHR
Medjeral-Thomas, N, Pickering, MC
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Complement and disease: better no factor H than bad factor H [PDF]
Within just a few minutes the complement system (1) can label a foreign cell that has invaded the bloodstream with millions of copies of C3b. Destruction or clearance of the C3b-coated invader quickly follows. C3b is the activated cleavage product of C3, an abundant serum protein.
P. Barlow
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Functional and structural characterization of mouse Factor H-related B protein unveils a novel dimerization domain shared by FHR-B and FH [PDF]
Factor H-related proteins (FHRs) are found in mice, but their equivalence to human FHRs remains uncertain. This study identifies three FHRs in mouse plasma (FHR-B, FHR-C, and FHR-E) and focuses on characterizing FHR-B.
Adrián Martín-Ambrosio Doménech+6 more
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Autoantibodies Against Factor B and Factor H Without Pathogenic Effects in a Patient with Immune Complex-Mediated Membranoproliferative Glomerulonephritis [PDF]
Background: Membranoproliferative glomerulonephritis (MPGN) is an umbrella term for chronic disorders affecting the glomeruli. MPGN is often accompanied by the presence of autoantibodies against complement components.
Alexandra T. Matola+6 more
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Microbes bind complement inhibitor factor H via a common site. [PDF]
To cause infections microbes need to evade host defense systems, one of these being the evolutionarily old and important arm of innate immunity, the alternative pathway of complement. It can attack all kinds of targets and is tightly controlled in plasma
T Meri+9 more
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Standardisation of the factor H autoantibody assay
The screening of all atypical haemolytic uraemic syndrome (aHUS) patients for factor H autoantibodies is best practice. However, there is no consensus assay for the reporting of factor H autoantibody titres. In this study, three European complement laboratories with expertise in the field of autoantibody testing address this by systematically ...
Christine Skerka+10 more
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Background and Objective: Neisseria meningitidis is a leading cause of meningitis and sepsis worldwide. The factor H binding protein (fHBP) is a key virulence factor of Neisseria meningitidis that is able to selectively bind to human factor H, the key ...
Fatemeh Yarian+3 more
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Mechanisms by which Factor H protects Trypanosoma cruzi from the alternative pathway of complement [PDF]
Chagas disease, a chronic disabling disease caused by the protozoan Trypanosoma cruzi, has no standardized treatment or preventative vaccine. The infective trypomastigote form of T.
Smrithi S. Menon+6 more
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Anti-factor H autoantibody-associated hemolytic uremic syndrome in an Argentine pediatric cohort
Aim: To describe the clinical characteristics and frequency of anti-factor H (FH) autoantibody-associated atypical hemolytic uremic syndrome (aHUS) in the first cohort of Argentine patients. Methods: The presence of anti-FH autoantibodies in 70 pediatric
Célia Dos Santos+7 more
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The Factor H protein family: The switchers of the complement alternative pathway
The factor H (FH) protein family is emerging as a complex network of proteins controlling the fate of the complement alternative pathway (AP) and dictating susceptibility to a wide range of diseases including infectious, inflammatory, autoimmune, and ...
Laura Lucientes-Continente+2 more
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