Endothelium structure and function in kidney health and disease

N Jourde-Chiche, F Fakhouri, L Dou, J Bellien… - Nature Reviews …, 2019 - nature.com
The kidney harbours different types of endothelia, each with specific structural and functional
characteristics. The glomerular endothelium, which is highly fenestrated and covered by a …

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) …

A single-nucleus RNA-sequencing pipeline to decipher the molecular anatomy and pathophysiology of human kidneys

BB Lake, S Chen, M Hoshi, N Plongthongkum… - Nature …, 2019 - nature.com
Defining cellular and molecular identities within the kidney is necessary to understand its
organization and function in health and disease. Here we demonstrate a reproducible …

[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 …

Statistical validation of rare complement variants provides insights into the molecular basis of atypical hemolytic uremic syndrome and C3 glomerulopathy

AJ Osborne, M Breno, NG Borsa, F Bu… - The Journal of …, 2018 - journals.aai.org
Atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G) are associated
with dysregulation and overactivation of the complement alternative pathway. Typically …

Complement inhibitors in clinical trials for glomerular diseases

PF Zipfel, T Wiech, R Rudnick, S Afonso… - Frontiers in …, 2019 - frontiersin.org
Defective complement action is a cause of several human glomerular diseases including
atypical hemolytic uremic syndrome (aHUS), anti-neutrophil cytoplasmic antibody mediated …

How I diagnose and treat atypical hemolytic uremic syndrome

F Fakhouri, N Schwotzer… - Blood, The Journal of …, 2023 - ashpublications.org
Our understanding and management of atypical hemolytic uremic syndrome (aHUS) have
dramatically improved in the last decade. aHUS has been established as a prototypic …

[HTML][HTML] Implications of genetic variation in the complement system in age-related macular degeneration

S de Jong, G Gagliardi, A Garanto, A de Breuk… - Progress in Retinal and …, 2021 - Elsevier
Age-related macular degeneration (AMD) is the main cause of vision loss among the elderly
in the Western world. While AMD is a multifactorial disease, the complement system was …

[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 …

Functional characterization of 105 factor H variants associated with aHUS: lessons for variant classification

H Martín Merinero, Y Zhang, E Arjona… - Blood, The Journal …, 2021 - ashpublications.org
Atypical hemolytic uremic syndrome (aHUS) is a life-threatening thrombotic microangiopathy
that can progress, when untreated, to end-stage renal disease. Most frequently, aHUS is …