Clinical promise of next-generation complement therapeutics

DC Mastellos, D Ricklin, JD Lambris - Nature reviews Drug discovery, 2019 - nature.com
The complement system plays a key role in pathogen immunosurveillance and tissue
homeostasis. However, subversion of its tight regulatory control can fuel a vicious cycle of …

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 …

[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 …

Tipping the balance: intricate roles of the complement system in disease and therapy

RB Pouw, D Ricklin - Seminars in Immunopathology, 2021 - Springer
The ability of the complement system to rapidly and broadly react to microbial intruders,
apoptotic cells and other threats by inducing forceful elimination responses is indispensable …

The immune system and kidney disease: basic concepts and clinical implications

C Kurts, U Panzer, HJ Anders, AJ Rees - Nature Reviews Immunology, 2013 - nature.com
The kidneys are frequently targeted by pathogenic immune responses against renal
autoantigens or by local manifestations of systemic autoimmunity. Recent studies in rodent …

[HTML][HTML] C3 glomerulopathy: consensus report

MC Pickering, VD D'agati, CM Nester, RJ Smith… - Kidney international, 2013 - Elsevier
C3 glomerulopathy is a recently introduced pathological entity whose original definition was
glomerular pathology characterized by C3 accumulation with absent or scanty …

Thrombotic microangiopathy in aHUS and beyond: clinical clues from complement genetics

F Fakhouri, V Frémeaux-Bacchi - Nature Reviews Nephrology, 2021 - nature.com
Studies of complement genetics have changed the landscape of thrombotic
microangiopathies (TMAs), particularly atypical haemolytic uraemic syndrome (aHUS) …

Exploring the genetic basis of early-onset chronic kidney disease

A Vivante, F Hildebrandt - Nature Reviews Nephrology, 2016 - nature.com
The primary causes of chronic kidney disease (CKD) in children differ from those of CKD in
adults. In the USA the most common diagnostic groups of renal disease that manifest before …

Genetic testing in the diagnosis of chronic kidney disease: recommendations for clinical practice

N Knoers, C Antignac, C Bergmann… - Nephrology Dialysis …, 2022 - academic.oup.com
The overall diagnostic yield of massively parallel sequencing–based tests in patients with
chronic kidney disease (CKD) is 30% for paediatric cases and 6–30% for adult cases. These …