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 …

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) …

Eculizumab discontinuation in children and adults with atypical hemolytic-uremic syndrome: a prospective multicenter study

F Fakhouri, M Fila, A Hummel, D Ribes… - Blood, The Journal …, 2021 - ashpublications.org
The optimal duration of eculizumab treatment in patients with atypical hemolytic uremic
syndrome (aHUS) remains poorly defined. We conducted a prospective national multicenter …

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 …

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 …

[HTML][HTML] Complement in secondary thrombotic microangiopathy

LMP Palma, M Sridharan, S Sethi - Kidney international reports, 2021 - Elsevier
Thrombotic microangiopathy (TMA) is a condition characterized by thrombocytopenia and
microangiopathic hemolytic anemia (MAHA) with varying degrees of organ damage in the …

Complementopathies and precision medicine

E Gavriilaki, RA Brodsky - The Journal of clinical …, 2020 - Am Soc Clin Investig
The renaissance of complement diagnostics and therapeutics has introduced precision
medicine into a widened field of complement-mediated diseases. In particular, complement …

Genetic justification of severe COVID-19 using a rigorous algorithm

E Gavriilaki, PG Asteris, T Touloumenidou… - Clinical …, 2021 - Elsevier
Recent studies suggest excessive complement activation in severe coronavirus disease-19
(COVID-19). The latter shares common characteristics with complement-mediated …

Membrane cofactor protein (MCP; CD46): deficiency states and pathogen connections

MK Liszewski, JP Atkinson - Current Opinion in Immunology, 2021 - Elsevier
Highlights•Membrane cofactor protein (MCP; CD46) protects host cells from complement
attack.•Rare variants leading to haploinsufficiency predispose to renal disease.•Subversion …

[HTML][HTML] Regulation of the complement system by pentraxins

K Haapasalo, S Meri - Frontiers in immunology, 2019 - frontiersin.org
The functions of pentraxins, like C-reactive protein (CRP), serum amyloid protein P (SAP)
and pentraxin-3 (PTX3), are to coordinate spatially and temporally targeted clearance of …