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 …

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

R Piras, M Breno, E Valoti, M Alberti… - Frontiers in …, 2021 - frontiersin.org
C3 Glomerulopathy (C3G) and Immune Complex-Mediated Membranoproliferative
glomerulonephritis (IC-MPGN) are rare diseases characterized by glomerular deposition of …

[HTML][HTML] Glomerular Complement Factor H–Related Protein 5 (FHR5) Is Highly Prevalent in C3 Glomerulopathy and Associated With Renal Impairment

NR Medjeral-Thomas, H Moffitt… - Kidney international …, 2019 - Elsevier
Introduction Therapeutic agents that target complement are increasingly available for
glomerular diseases. However, the mechanisms linking glomerular complement deposition …

Complement Factor H related protein 1 and immune inflammatory disorders

X Li, J Zong, S Si - Molecular immunology, 2022 - Elsevier
Human complement Factor H-related protein 1 (FHR-1) is one of complement Factor H-
related proteins (FHRs) and plays an important role in innate immunity. In particular, FHR-1 …

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

A Tortajada, H Yébenes… - The Journal of …, 2013 - Am Soc Clin Investig
C3 glomerulopathies (C3G) are a group of severe renal diseases with distinct patterns of
glomerular inflammation and C3 deposition caused by complement dysregulation. Here we …

Factor H–related protein 1 drives disease susceptibility and prognosis in C3 glomerulopathy

B Márquez-Tirado, J Gutiérrez-Tenorio… - Journal of the …, 2022 - journals.lww.com
Background C3 glomerulopathy (C3G) is a heterogeneous group of chronic renal diseases
characterized predominantly by glomerular C3 deposition and complement dysregulation …

Gain-of-function factor H–related 5 protein impairs glomerular complement regulation resulting in kidney damage

TH Malik, DP Gitterman, DP Lavin… - Proceedings of the …, 2021 - National Acad Sciences
Genetic variation within the factor H–related (FHR) genes is associated with the complement-
mediated kidney disease, C3 glomerulopathy (C3G). There is no definitive treatment for …

[HTML][HTML] Glomerular complement factor H–related protein 5 is associated with histologic injury in immunoglobulin A nephropathy

W Guo, L Sun, H Dong, G Wang, X Xu, Z Zhao… - Kidney International …, 2021 - Elsevier
Introduction Immunoglobulin A nephrology (IgAN), characterized by co-deposition of IgA and
complement components, is an activation of complement system involved disease. Factor H …

[HTML][HTML] Factor H–related protein 5 interacts with pentraxin 3 and the extracellular matrix and modulates complement activation

ÁI Csincsi, A Kopp, M Zöldi, Z Bánlaki… - The Journal of …, 2015 - journals.aai.org
The physiological roles of the factor H (FH)-related proteins are controversial and poorly
understood. Based on genetic studies, FH-related protein 5 (CFHR5) is implicated in …

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

X Xiao, C Ghossein, A Tortajada, Y Zhang, N Meyer… - Molecular …, 2016 - Elsevier
Abstract C3 glomerulopathy (C3G) is an ultra-rare complement-mediated renal disease
characterized histologically by the predominance of C3 deposition within in the glomerulus …