Factor H–Related Protein 1 Drives Disease Susceptibility and Prognosis in C3 Glomerulopathy

The molecular bases for the association of FHR-1 with atypical hemolytic uremic syndrome and other diseases.

(2021) Hector Martin Merinero et al.   BLOOD

Deregulation of Factor H by Factor H-Related Protein 1 Depends on Sialylation of Host Surfaces

(2021) Arthur Dopler et al.   Frontiers in Immunology

Common haplotypes at the CFH locus and low-frequency variants in CFHR2 and CFHR5 associate with systemic FHR concentrations and age-related macular degeneration

(2021) Laura Lorés-Motta et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Functional characterization of 105 Factor H variants associated with atypical HUS: lessons for variant classification.

(2021) Hector Martin Merinero et al.   BLOOD

Selective Binding of Heparin/Heparan Sulfate Oligosaccharides to Factor H and Factor H-Related Proteins: Therapeutic Potential for C3 Glomerulopathies

(2021) Markus A. Loeven et al.   Frontiers in Immunology

CFH and CFHR Copy Number Variations in C3 Glomerulopathy and Immune Complex-Mediated Membranoproliferative Glomerulonephritis

(2021) Rossella Piras et al.   Frontiers in Genetics

Mycophenolate Mofetil in C3 Glomerulopathy and Pathogenic Drivers of the Disease

(2020) Fernando Caravaca-Fontán et al.   Clinical Journal of the American Society of Nephrology

C3 glomerulopathy — understanding a rare complement-driven renal disease

(2019) Richard J. H. Smith et al.   Nature Reviews Nephrology

Nephritic Factors: An Overview of Classification, Diagnostic Tools and Clinical Associations

(2019) Fernando Corvillo et al.   Frontiers in Immunology

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

(2018) Pilar Sánchez-Corral et al.   Frontiers in Immunology

Unraveling the Molecular Mechanisms Underlying Complement Dysregulation by Nephritic Factors in C3G and IC-MPGN

(2018) Roberta Donadelli et al.   Frontiers in 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

Familial C3 glomerulonephritis caused by a novel CFHR5-CFHR2 fusion gene

(2016) Xue Xiao et al.   MOLECULAR IMMUNOLOGY

Mapping interactions between complement C3 and regulators using mutations in atypical hemolytic uremic syndrome

(2015) E. C. Schramm et al.   BLOOD

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

Factor H-related proteins determine complement-activating surfaces

(2015) Mihály Józsi et al.   TRENDS IN IMMUNOLOGY

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

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

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

C3 glomerulopathy: consensus report

(2013) Matthew C. Pickering et al.   KIDNEY INTERNATIONAL

Toward a working definition of C3 glomerulopathy by immunofluorescence

(2013) Jean Hou et al.   KIDNEY INTERNATIONAL

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

Causes of Alternative Pathway Dysregulation in Dense Deposit Disease

(2012) Y. Zhang et al.   Clinical Journal of the American Society of Nephrology

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

Genome-wide association study identifies susceptibility loci for IgA nephropathy

(2011) Ali G Gharavi et al.   NATURE GENETICS

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

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

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

(2009) S. Heinen et al.   BLOOD