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 …
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) …
microangiopathies (TMAs), particularly atypical haemolytic uraemic syndrome (aHUS) …
A single-nucleus RNA-sequencing pipeline to decipher the molecular anatomy and pathophysiology of human kidneys
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 …
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 …
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
Atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G) are associated
with dysregulation and overactivation of the complement alternative pathway. Typically …
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 …
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 …
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
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 …
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 …
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 …
that can progress, when untreated, to end-stage renal disease. Most frequently, aHUS is …