Self-Damage Caused by Dysregulation of the Complement Alternative Pathway: Relevance of the Factor H Protein Family

FHR5 Binds to Laminins, Uses Separate C3b and Surface-Binding Sites, and Activates Complement on Malondialdehyde-Acetaldehyde Surfaces

(2018) Ramona B. Rudnick et al.   JOURNAL OF IMMUNOLOGY

Recurrent structural variation, clustered sites of selection, and disease risk for the complement factor H (CFH) gene family

(2018) Stuart Cantsilieris et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Myeloperoxidase influences the complement regulatory activity of complement factor H

(2018) Su-Fang Chen et al.   RHEUMATOLOGY

Complement factor H family proteins in their non-canonical role as modulators of cellular functions

(2018) Mihály Józsi et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

C-reactive protein and pentraxin-3 binding of factor H-like protein 1 differs from complement factor H: implications for retinal inflammation

(2018) Maurice Swinkels et al.   Scientific Reports

High Complement Factor H-Related (FHR)-3 Levels Are Associated With the Atypical Hemolytic-Uremic Syndrome-Risk Allele CFHR3*B

(2018) Richard B. Pouw et al.   Frontiers in Immunology

Complement Factor H-Related Protein 4A Is the Dominant Circulating Splice Variant of CFHR4

(2018) Richard B. Pouw et al.   Frontiers in Immunology

Complement Factor H Inhibits CD47-Mediated Resolution of Inflammation

(2017) Bertrand Calippe et al.   IMMUNITY

Modulation of the Alternative Pathway of Complement by Murine Factor H–Related Proteins

(2017) Alexandra H. Antonioli et al.   JOURNAL OF IMMUNOLOGY

FHR-1 Binds to C-Reactive Protein and Enhances Rather than Inhibits Complement Activation

(2017) Ádám I. Csincsi et al.   JOURNAL OF IMMUNOLOGY

Factor H Competitor Generated by Gene Conversion Events Associates with Atypical Hemolytic Uremic Syndrome

(2017) Elena Goicoechea de Jorge et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Circulating complement factor H–related proteins 1 and 5 correlate with disease activity in IgA nephropathy

(2017) Nicholas R. Medjeral-Thomas et al.   KIDNEY INTERNATIONAL

Elevated factor H–related protein 1 and factor H pathogenic variants decrease complement regulation in IgA nephropathy

(2017) Agustín Tortajada et al.   KIDNEY INTERNATIONAL

Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a “Kidney Disease: Improving Global Outcomes” (KDIGO) Controversies Conference

(2017) Timothy H.J. Goodship et al.   KIDNEY INTERNATIONAL

Novel duplication of the FHRs dimerization domain associated with C3G

(2017) Agustín Tortajada et al.   MOLECULAR IMMUNOLOGY

The complement system in age-related macular degeneration: A review of rare genetic variants and implications for personalized treatment

(2017) Maartje J. Geerlings et al.   MOLECULAR IMMUNOLOGY

The renaissance of complement therapeutics

(2017) Daniel Ricklin et al.   Nature Reviews Nephrology

Regulator-dependent mechanisms of C3b processing by factor I allow differentiation of immune responses

(2017) Xiaoguang Xue et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

A novel, multiplexed targeted mass spectrometry assay for quantification of complement factor H (CFH) variants and CFH-related proteins 1-5 in human plasma

(2017) Pingbo Zhang et al.   PROTEOMICS

Complement system biomarkers in first episode psychosis

(2017) Maja Kopczynska et al.   SCHIZOPHRENIA RESEARCH

Regulator-dependent mechanisms of C3b processing by factor I allow differentiation of immune responses

(2017) Xiaoguang Xue et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Factor H Family Proteins in Complement Evasion of Microorganisms

(2017) Mihály Józsi   Frontiers in Immunology

The Murine Factor H-Related Protein FHR-B Promotes Complement Activation

(2017) Marcell Cserhalmi et al.   Frontiers in Immunology

Factor H-Related (FHR)-1 and FHR-2 Form Homo- and Heterodimers, while FHR-5 Circulates Only As Homodimer in Human Plasma

(2017) Anna E. van Beek et al.   Frontiers in Immunology

Disturbed sialic acid recognition on endothelial cells and platelets in complement attack causes atypical hemolytic uremic syndrome

(2016) S. Hyvarinen et al.   BLOOD

Self-nonself discrimination by the complement system

(2016) Seppo Meri   FEBS LETTERS

Complement in removal of the dead - balancing inflammation

(2016) Myriam Martin et al.   IMMUNOLOGICAL REVIEWS

Endothelial cells: source, barrier, and target of defensive mediators

(2016) Lubka T. Roumenina et al.   IMMUNOLOGICAL REVIEWS

FHR3 Blocks C3d-Mediated Coactivation of Human B Cells

(2016) Denise Buhlmann et al.   JOURNAL OF IMMUNOLOGY

The Complement Inhibitor Factor H Generates an Anti-Inflammatory and Tolerogenic State in Monocyte-Derived Dendritic Cells

(2016) Rut Olivar et al.   JOURNAL OF IMMUNOLOGY

Complement factor H modulates the activation of human neutrophil granulocytes and the generation of neutrophil extracellular traps

(2016) Andrea E. Schneider et al.   MOLECULAR IMMUNOLOGY

Heterogeneity but individual constancy of epitopes, isotypes and avidity of factor H autoantibodies in atypical hemolytic uremic syndrome

(2016) Pilar Nozal et al.   MOLECULAR IMMUNOLOGY

Factor H inhibits complement activation induced by liposomal and micellar drugs and the therapeutic antibody rituximab in vitro

(2016) Tamás Mészáros et al.   Nanomedicine-Nanotechnology Biology and Medicine

Thrombomodulin enhances complement regulation through strong affinity interactions with factor H and C3b-Factor H complex

(2016) M. Heurich et al.   THROMBOSIS RESEARCH

Dual modulating functions of thrombomodulin in the alternative complement pathway

(2016) Koichiro Tateishi et al.   BioScience Trends

Complement Regulator FHR-3 Is Elevated either Locally or Systemically in a Selection of Autoimmune Diseases

(2016) Nicole Schäfer et al.   Frontiers in Immunology

Complement Factor H-Related Protein 3 Serum Levels Are Low Compared to Factor H and Mainly Determined by Gene Copy Number Variation in CFHR3

(2016) Richard B. Pouw et al.   PLoS One

Mapping the Complement Factor H-Related Protein 1 (CFHR1):C3b/C3d Interactions

(2016) Jonathan P. Hannan et al.   PLoS One

Secreted aspartic protease 2 of Candida albicans inactivates factor H and the macrophage factor H-receptors CR3 (CD11b/CD18) and CR4 (CD11c/CD18)

(2015) Eliška Svoboda et al.   IMMUNOLOGY LETTERS

The Major Autoantibody Epitope on Factor H in Atypical Hemolytic Uremic Syndrome Is Structurally Different from Its Homologous Site in Factor H-related Protein 1, Supporting a Novel Model for Induction of Autoimmunity in This Disease

(2015) Arnab Bhattacharjee et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Complement Factor H Binds to Human Serum Apolipoprotein E and Mediates Complement Regulation on High Density Lipoprotein Particles

(2015) Karita Haapasalo et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Anti–Factor H Autoantibodies in C3 Glomerulopathies and in Atypical Hemolytic Uremic Syndrome: One Target, Two Diseases

(2015) Caroline Blanc et al.   JOURNAL OF IMMUNOLOGY

Factor H–Related Protein 5 Interacts with Pentraxin 3 and the Extracellular Matrix and Modulates Complement Activation

(2015) Ádám I. Csincsi et al.   JOURNAL OF IMMUNOLOGY

A De Novo Deletion in the Regulators of Complement Activation Cluster Producing a Hybrid Complement Factor H/Complement Factor H-Related 3 Gene in Atypical Hemolytic Uremic Syndrome

(2015) R. C. Challis et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Current Understanding of the Role of Complement in IgA Nephropathy

(2015) N. Maillard et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Complement Factor H-Related 5-Hybrid Proteins Anchor Properdin and Activate Complement at Self-Surfaces

(2015) Q. Chen et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Molecular Basis of Factor H R1210C Association with Ocular and Renal Diseases

(2015) S. Recalde et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Complement factor H, FHR-3 and FHR-1 variants associate in an extended haplotype conferring increased risk of atypical hemolytic uremic syndrome

(2015) Maria E. Bernabéu-Herrero et al.   MOLECULAR IMMUNOLOGY

Atypical aHUS: State of the art

(2015) Carla M. Nester et al.   MOLECULAR IMMUNOLOGY

A large genome-wide association study of age-related macular degeneration highlights contributions of rare and common variants

(2015) Lars G Fritsche et al.   NATURE GENETICS

Factor H-related proteins determine complement-activating surfaces

(2015) Mihály Józsi et al.   TRENDS IN IMMUNOLOGY

Binding of the Long Pentraxin PTX3 to Factor H: Interacting Domains and Function in the Regulation of Complement Activation

(2014) L. Deban et al.   JOURNAL OF IMMUNOLOGY

A New Map of Glycosaminoglycan and C3b Binding Sites on Factor H

(2014) C. Q. Schmidt et al.   JOURNAL OF IMMUNOLOGY

Identification of Factor H–like Protein 1 as the Predominant Complement Regulator in Bruch’s Membrane: Implications for Age-Related Macular Degeneration

(2014) Simon J. Clark et al.   JOURNAL OF IMMUNOLOGY

A Novel Atypical Hemolytic Uremic Syndrome-Associated Hybrid CFHR1/CFH Gene Encoding a Fusion Protein That Antagonizes Factor H-Dependent Complement Regulation

(2014) E. Valoti et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Variants in Complement Factor H and Complement Factor H-Related Protein Genes, CFHR3 and CFHR1, Affect Complement Activation in IgA Nephropathy

(2014) L. Zhu et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

An ELISA assay with two monoclonal antibodies allows the estimation of free factor H and identifies patients with acquired deficiency of this complement regulator

(2014) Pilar Nozal et al.   MOLECULAR IMMUNOLOGY

Structural basis for sialic acid–mediated self-recognition by complement factor H

(2014) Bärbel S Blaum et al.   Nature Chemical Biology

Competition between antagonistic complement factors for a single protein on N. meningitidis rules disease susceptibility

(2014) Joseph JE Caesar et al.   eLife

Human Complement Factor H Is a Reductase for Large Soluble von Willebrand Factor Multimers—Brief Report

(2013) Leticia Nolasco et al.   ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY

The interaction between factor H and VWF increases factor H cofactor activity and regulates VWF prothrombotic status

(2013) J. Rayes et al.   BLOOD

Complement activation by heme as a secondary hit for atypical hemolytic uremic syndrome

(2013) M. Frimat et al.   BLOOD

Genetic influences on plasma CFH and CFHR1 concentrations and their role in susceptibility to age-related macular degeneration

(2013) Morad Ansari et al.   HUMAN MOLECULAR GENETICS

Recognition of Malondialdehyde-modified Proteins by the C Terminus of Complement Factor H Is Mediated via the Polyanion Binding Site and Impaired by Mutations Found in Atypical Hemolytic Uremic Syndrome

(2013) Satu Hyvärinen et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

C3 glomerulopathy–associated CFHR1 mutation alters FHR oligomerization and complement regulation

(2013) Agustín Tortajada et al.   JOURNAL OF CLINICAL INVESTIGATION

Complement factor H–related hybrid protein deregulates complement in dense deposit disease

(2013) Qian Chen et al.   JOURNAL OF CLINICAL INVESTIGATION

Distribution and determinants of circulating complement factor H concentration determined by a high-throughput immunonephelometric assay

(2013) Reecha Sofat et al.   JOURNAL OF IMMUNOLOGICAL METHODS

Tissue-Specific Host Recognition by Complement Factor H Is Mediated by Differential Activities of Its Glycosaminoglycan-Binding Regions

(2013) S. J. Clark et al.   JOURNAL OF IMMUNOLOGY

An Engineered Construct Combining Complement Regulatory and Surface-Recognition Domains Represents a Minimal-Size Functional Factor H

(2013) M. Hebecker et al.   JOURNAL OF IMMUNOLOGY

A novel CFHR5 fusion protein causes C3 glomerulopathy in a family without Cypriot ancestry

(2013) Nicholas Medjeral-Thomas et al.   KIDNEY INTERNATIONAL

Complement factor H related proteins (CFHRs)

(2013) Christine Skerka et al.   MOLECULAR IMMUNOLOGY

A novel hybrid CFHR1/CFH gene causes atypical hemolytic uremic syndrome

(2013) Stephen J. Eyler et al.   PEDIATRIC NEPHROLOGY

Determining the Population Frequency of the CFHR3/CFHR1 Deletion at 1q32

(2013) Lucy V. Holmes et al.   PLoS One

The Interaction between Factor H and Von Willebrand Factor

(2013) Shuju Feng et al.   PLoS One

Human Factor H-Related Protein 2 (CFHR2) Regulates Complement Activation

(2013) Hannes U. Eberhardt et al.   PLoS One

Dimerization of complement factor H-related proteins modulates complement activation in vivo

(2013) E. Goicoechea de Jorge et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Proteoglycan Glycomatrix: A Sugar Microenvironment Essential for Complement Regulation

(2013) Simon J. Clark et al.   Frontiers in Immunology

Acute Presentation and Persistent Glomerulonephritis Following Streptococcal Infection in a Patient With Heterozygous Complement Factor H–Related Protein 5 Deficiency

(2012) Katherine A. Vernon et al.   AMERICAN JOURNAL OF KIDNEY DISEASES

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

(2012) Mario Hebecker et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Overall Neutralization of Complement Factor H by Autoantibodies in the Acute Phase of the Autoimmune Form of Atypical Hemolytic Uremic Syndrome

(2012) C. Blanc et al.   JOURNAL OF IMMUNOLOGY

Atypical Hemolytic Uremic Syndrome-Associated Variants and Autoantibodies Impair Binding of Factor H and Factor H-Related Protein 1 to Pentraxin 3

(2012) A. Kopp et al.   JOURNAL OF IMMUNOLOGY

A Hybrid CFHR3-1 Gene Causes Familial C3 Glomerulopathy

(2012) T. H. Malik et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies

(2012) Aude Servais et al.   KIDNEY INTERNATIONAL

Factor H autoantibodies in membranoproliferative glomerulonephritis

(2012) Timothy H.J. Goodship et al.   MOLECULAR IMMUNOLOGY

Factor H autoantibodies and deletion of Complement Factor H-Related protein-1 in rheumatic diseases in comparison to atypical hemolytic uremic syndrome

(2012) Anna Foltyn Zadura et al.   ARTHRITIS RESEARCH & THERAPY

A novel hybrid CFH/CFHR3 gene generated by a microhomology-mediated deletion in familial atypical hemolytic uremic syndrome

(2011) N. J. Francis et al.   BLOOD

Familial C3 Glomerulopathy Associated with CFHR5 Mutations: Clinical Characteristics of 91 Patients in 16 Pedigrees

(2011) Y. Athanasiou et al.   Clinical Journal of the American Society of Nephrology

The Pathophysiology of IgA Nephropathy

(2011) H. Suzuki et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Factor H-related protein 1 neutralizes anti-factor H autoantibodies in autoimmune hemolytic uremic syndrome

(2011) Stefanie Strobel et al.   KIDNEY INTERNATIONAL

Complement factor H binds malondialdehyde epitopes and protects from oxidative stress

(2011) David Weismann et al.   NATURE

A rare penetrant mutation in CFH confers high risk of age-related macular degeneration

(2011) Soumya Raychaudhuri et al.   NATURE GENETICS

Genome-wide association study identifies susceptibility loci for IgA nephropathy

(2011) Ali G Gharavi et al.   NATURE GENETICS

Structural basis for engagement by complement factor H of C3b on a self surface

(2011) Hugh P Morgan et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Human Pentraxin 3 Binds to the Complement Regulator C4b-Binding Protein

(2011) Anne Braunschweig et al.   PLoS One

A 32 kb Critical Region Excluding Y402H in CFH Mediates Risk for Age-Related Macular Degeneration

(2011) Theru A. Sivakumaran et al.   PLoS One

Dual interaction of factor H with C3d and glycosaminoglycans in host-nonhost discrimination by complement

(2011) T. Kajander et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Association of Genetic Variants in Complement Factor H and Factor H-Related Genes with Systemic Lupus Erythematosus Susceptibility

(2011) Jian Zhao et al.   PLoS Genetics

Recurrence of Complement Factor H-Related Protein 5 Nephropathy in a Renal Transplant

(2010) K. A. Vernon et al.   AMERICAN JOURNAL OF TRANSPLANTATION

Pentraxins in innate immunity: lessons from PTX3

(2010) Livija Deban et al.   CELL AND TISSUE RESEARCH

Regulation of C3 and factor H synthesis of human glomerular mesangial cells by IL-1 and interferon-gamma

(2010) M. E. A. DOBBELSTEEN et al.   CLINICAL AND EXPERIMENTAL IMMUNOLOGY

An imbalance of human complement regulatory proteins CFHR1, CFHR3 and factor H influences risk for age-related macular degeneration (AMD)

(2010) Lars G. Fritsche et al.   HUMAN MOLECULAR GENETICS

Impaired Binding of the Age-related Macular Degeneration-associated Complement Factor H 402H Allotype to Bruch's Membrane in Human Retina

(2010) Simon J. Clark et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Angiogenic Inhibitor Long Pentraxin PTX3 Forms an Asymmetric Octamer with Two Binding Sites for FGF2

(2010) Antonio Inforzato et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Heterocomplexes of Mannose-binding Lectin and the Pentraxins PTX3 or Serum Amyloid P Component Trigger Cross-activation of the Complement System

(2010) Ying Jie Ma et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Complement Regulator Factor H Mediates a Two-step Uptake ofStreptococcus pneumoniaeby Human Cells

(2010) Vaibhav Agarwal et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Identification of a mutation in complement factor H-related protein 5 in patients of Cypriot origin with glomerulonephritis

(2010) Daniel P Gale et al.   LANCET

A novel deletion in the RCA gene cluster causes atypical hemolytic uremic syndrome

(2010) T. K. Maga et al.   NEPHROLOGY DIALYSIS TRANSPLANTATION

Anti-Factor H Autoantibody–Associated Hemolytic Uremic Syndrome: Review of Literature of the Autoimmune Form of HUS

(2010) Marie-Agnès Dragon-Durey et al.   SEMINARS IN THROMBOSIS AND HEMOSTASIS

An Integrated View of Humoral Innate Immunity: Pentraxins as a Paradigm

(2009) Barbara Bottazzi et al.   Annual Review of Immunology

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

(2009) S. Heinen et al.   BLOOD

Association of factor H autoantibodies with deletions of CFHR1, CFHR3, CFHR4, and with mutations in CFH, CFI, CD46, and C3 in patients with atypical hemolytic uremic syndrome

(2009) I. Moore et al.   BLOOD

Characterization of complement factor H-related (CFHR) proteins in plasma reveals novel genetic variations of CFHR1 associated with atypical hemolytic uremic syndrome

(2009) C. Abarrategui-Garrido et al.   BLOOD

Monomeric CRP contributes to complement control in fluid phase and on cellular surfaces and increases phagocytosis by recruiting factor H

(2009) M Mihlan et al.   CELL DEATH AND DIFFERENTIATION

The disease-protective complement factor H allotypic variant Ile62 shows increased binding affinity for C3b and enhanced cofactor activity

(2009) A. Tortajada et al.   HUMAN MOLECULAR GENETICS

Annexin-II, DNA, and Histones Serve as Factor H Ligands on the Surface of Apoptotic Cells

(2009) Jonatan Leffler et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Complement Factor H Binds at Two Independent Sites to C-reactive Protein in Acute Phase Concentrations

(2009) Azubuike I. Okemefuna et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Factor H and Factor H-Related Protein 1 Bind to Human Neutrophils via Complement Receptor 3, Mediate Attachment to Candida albicans, and Enhance Neutrophil Antimicrobial Activity

(2009) J. Losse et al.   JOURNAL OF IMMUNOLOGY

The high frequency of complement factor H related CFHR1 gene deletion is restricted to specific subgroups of patients with atypical haemolytic uraemic syndrome

(2009) M-A Dragon-Durey et al.   JOURNAL OF MEDICAL GENETICS

Molecular basis of C-reactive protein binding and modulation of complement activation by factor H-related protein 4

(2009) Mario Hebecker et al.   MOLECULAR IMMUNOLOGY

Functional analyses indicate a pathogenic role of factor H autoantibodies in atypical haemolytic uraemic syndrome

(2009) S. Strobel et al.   NEPHROLOGY DIALYSIS TRANSPLANTATION

Thrombomodulin Mutations in Atypical Hemolytic–Uremic Syndrome

(2009) Mieke Delvaeye et al.   NEW ENGLAND JOURNAL OF MEDICINE

Atypical Hemolytic–Uremic Syndrome

(2009) Marina Noris et al.   NEW ENGLAND JOURNAL OF MEDICINE

Complement activation and inhibition: a delicate balance

(2009) A.P. Sjöberg et al.   TRENDS IN IMMUNOLOGY

Complement Factor H Binds to Denatured Rather than to Native Pentameric C-reactive Protein

(2008) Svetlana Hakobyan et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Human complement factor H-related protein 4 binds and recruits native pentameric C-reactive protein to necrotic cells

(2008) Michael Mihlan et al.   MOLECULAR IMMUNOLOGY

Complement evasion by human pathogens

(2008) John D. Lambris et al.   NATURE REVIEWS MICROBIOLOGY