C3 glomerulopathy—understanding a rare complement-driven renal disease

RJH Smith, GB Appel, AM Blom, HT Cook… - Nature reviews …, 2019 - nature.com
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 …

Complement system part I–molecular mechanisms of activation and regulation

NS Merle, SE Church, V Fremeaux-Bacchi… - Frontiers in …, 2015 - frontiersin.org
Complement is a complex innate immune surveillance system, playing a key role in defense
against pathogens and in host homeostasis. The complement system is initiated by …

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

THJ Goodship, HT Cook, F Fakhouri, FC Fervenza… - Kidney international, 2017 - Elsevier
In both atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G)
complement plays a primary role in disease pathogenesis. Herein we report the outcome of …

Complement system part II: role in immunity

NS Merle, R Noe, L Halbwachs-Mecarelli… - Frontiers in …, 2015 - frontiersin.org
The complement system has been considered for a long time as a simple lytic cascade,
aimed to kill bacteria infecting the host organism. Nowadays, this vision has changed and it …

[HTML][HTML] Overview of complement activation and regulation

M Noris, G Remuzzi - Seminars in nephrology, 2013 - Elsevier
Complement is an important component of the innate immune system that is crucial for
defense from microbial infections and for clearance of immune complexes and injured cells …

Complement activation, regulation, and molecular basis for complement‐related diseases

G Bajic, SE Degn, S Thiel, GR Andersen - The EMBO journal, 2015 - embopress.org
The complement system is an essential element of the innate immune response that
becomes activated upon recognition of molecular patterns associated with microorganisms …

Current understanding of the role of complement in IgA nephropathy

N Maillard, RJ Wyatt, BA Julian, K Kiryluk… - Journal of the …, 2015 - journals.lww.com
Complement activation has a role in the pathogenesis of IgA nephropathy, an autoimmune
disease mediated by pathogenic immune complexes consisting of galactose-deficient IgA1 …

Complement and its receptors: new insights into human disease

VM Holers - Annual review of immunology, 2014 - annualreviews.org
Although new activation and regulatory mechanisms are still being identified, the basic
architecture of the complement system has been known for decades. Two major roles of …

[HTML][HTML] New developments in the genetics, pathogenesis, and therapy of IgA nephropathy

R Magistroni, VD D'Agati, GB Appel, K Kiryluk - Kidney international, 2015 - Elsevier
Recent years have brought notable progress in the field of IgA nephropathy. Here, we
highlight important new directions and latest developments, including successful discovery …

Atypical aHUS: state of the art

CM Nester, T Barbour, SR de Cordoba… - Molecular …, 2015 - Elsevier
Tremendous advances in our understanding of the thrombotic microangiopathies (TMAs)
have revealed distinct disease mechanisms within this heterogeneous group of diseases. As …