C3 glomerulopathy—understanding a rare complement-driven renal disease
The C3 glomerulopathies are a group of rare kidney diseases characterized by complement
dysregulation occurring in the fluid phase and in the glomerular microenvironment, which …
dysregulation occurring in the fluid phase and in the glomerular microenvironment, which …
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 …
fate of the complement alternative pathway (AP) and dictating susceptibility to a wide range …
Genetic variability shapes the alternative pathway complement activity and predisposition to complement‐related diseases
S Rodríguez de Córdoba - Immunological Reviews, 2023 - Wiley Online Library
The implementation of next‐generation sequencing technologies has provided a sharp
picture of the genetic variability in the components and regulators of the alternative pathway …
picture of the genetic variability in the components and regulators of the alternative pathway …
Diseases of complement dysregulation—an overview
EKS Wong, D Kavanagh - Seminars in immunopathology, 2018 - Springer
Atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G), and paroxysmal
nocturnal hemoglobinuria (PNH) are prototypical disorders of complement dysregulation …
nocturnal hemoglobinuria (PNH) are prototypical disorders of complement dysregulation …
[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 …
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 …
comprising the complement genes CFHR1, CFHR2, CFHR3, CFHR4, CFHR5, and Factor H …
A family affair: addressing the challenges of factor H and the related proteins
F Poppelaars, E Goicoechea de Jorge… - Frontiers in …, 2021 - frontiersin.org
Inflammation is a common denominator of diseases. The complement system, an intrinsic
part of the innate immune system, is a key driver of inflammation in numerous disorders …
part of the innate immune system, is a key driver of inflammation in numerous disorders …
[HTML][HTML] Pitfalls in complement analysis: A systematic literature review of assessing complement activation
RJ Brandwijk, MAHM Michels, M van Rossum… - Frontiers in …, 2022 - frontiersin.org
Background The complement system is an essential component of our innate defense and
plays a vital role in the pathogenesis of many diseases. Assessment of complement …
plays a vital role in the pathogenesis of many diseases. Assessment of complement …
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 …
mediated kidney disease, C3 glomerulopathy (C3G). There is no definitive treatment for …
[HTML][HTML] Complement dysregulation in glomerulonephritis
Glomerulonephritis (GN) refers to a group of renal diseases affecting the glomeruli due to
the damage mediated by immunological mechanisms. A large proportion of the disease …
the damage mediated by immunological mechanisms. A large proportion of the disease …