The Factor H protein family: The switchers of the complement alternative pathway

L Lucientes‐Continente… - Immunological …, 2023 - Wiley Online Library
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 …

[HTML][HTML] Regulation of regulators: Role of the complement factor H-related proteins

M Cserhalmi, A Papp, B Brandus, B Uzonyi… - Seminars in …, 2019 - Elsevier
The complement system, while being an essential and very efficient effector component of
innate immunity, may cause damage to the host and result in various inflammatory …

CFHR gene variations provide insights in the pathogenesis of the kidney diseases atypical hemolytic uremic syndrome and C3 glomerulopathy

PF Zipfel, T Wiech, ED Stea… - Journal of the American …, 2020 - journals.lww.com
Sequence and copy number variations in the human CFHR–Factor H gene cluster
comprising the complement genes CFHR1, CFHR2, CFHR3, CFHR4, CFHR5, and Factor H …

A proteogenomic signature of age-related macular degeneration in blood

V Emilsson, EF Gudmundsson, T Jonmundsson… - Nature …, 2022 - nature.com
Age-related macular degeneration (AMD) is one of the most common causes of visual
impairment in the elderly, with a complex and still poorly understood etiology. Whole …

Serum FHR1 binding to necrotic-type cells activates monocytic inflammasome and marks necrotic sites in vasculopathies

S Irmscher, SR Brix, SLH Zipfel, LD Halder… - Nature …, 2019 - nature.com
Persistent inflammation is a hallmark of many human diseases, including anti-neutrophil
cytoplasmic antibody-associated vasculitis (AAV) and atherosclerosis. Here, we describe a …

Self-damage caused by dysregulation of the complement alternative pathway: relevance of the factor H protein family

P Sánchez-Corral, RB Pouw… - Frontiers in …, 2018 - frontiersin.org
The alternative pathway is a continuously active surveillance arm of the complement system,
and it can also enhance complement activation initiated by the classical and the lectin …

Complement factor H-related proteins FHR1 and FHR5 interact with extracellular matrix ligands, reduce factor H regulatory activity and enhance complement …

A Papp, K Papp, B Uzonyi, M Cserhalmi… - Frontiers in …, 2022 - frontiersin.org
Components of the extracellular matrix (ECM), when exposed to body fluids may promote
local complement activation and inflammation. Pathologic complement activation at the …

Complement factor H-related 3 enhanced inflammation and complement activation in human RPE cells

N Schäfer, A Rasras, DM Ormenisan… - Frontiers in …, 2021 - frontiersin.org
Complement Factor H-Related 3 (FHR-3) is a major regulator of the complement system,
which is associated with different diseases, such as age-related macular degeneration …

Complement catalyzing glomerular diseases

PF Zipfel, T Wiech, HJ Gröne, C Skerka - Cell and Tissue Research, 2021 - Springer
Complement is an evolutionarily conserved system which is important in the defense against
microorganisms and also in the elimination of modified or necrotic elements of the body …

FHR-5 Serum Levels and CFHR5 Genetic Variations in Patients With Immune Complex-Mediated Membranoproliferative Glomerulonephritis and C3-Glomerulopathy

N Garam, M Cserhalmi, Z Prohászka… - Frontiers in …, 2021 - frontiersin.org
Background Factor H-related protein 5 (FHR-5) is a member of the complement Factor H
protein family. Due to the homology to Factor H, the main complement regulator of the …