Associated Papers
The Inhibitory Effects of a Factor B-Binding DNA Aptamer Family Supersede the Gain of Function of Factor B Variants Associated with Atypical Hemolytic Uremic Syndrome.
Duan H et al.
Journal of Immunology (baltimore, Md. : 1950), 2024
Genetic variability shapes the alternative pathway complement activity and predisposition to complement-related diseases.
Rodríguez de Córdoba S
Immunological Reviews, 2023
Novel Complement Factor B Gene Mutation Identified in a Kidney Transplant Recipient with a Shiga Toxin-Triggered Episode of Thrombotic Microangiopathy.
Korzycka J et al.
The American Journal of Case Reports, 2022
Modeling C3 glomerulopathies: C3 convertase regulation on an extracellular matrix surface.
Pisarenka S et al.
Frontiers in Immunology, 2022
Factor D Inhibition Blocks Complement Activation Induced by Mutant Factor B Associated With Atypical Hemolytic Uremic Syndrome and Membranoproliferative Glomerulonephritis.
Aradottir SS et al.
Frontiers in Immunology, 2021
Mutation of complement factor B causing massive fluid-phase dysregulation of the alternative complement pathway can result in atypical hemolytic uremic syndrome.
Zhang Y et al.
Kidney International, 2020
Atypical hemolytic uremic syndrome associated with a factor B genetic variant and fluid-phase complement activation: an exception to the rule?
Noris M et al.
Kidney International, 2020
The role of ADAMTS-13 activity and complement mutational analysis in differentiating acute thrombotic microangiopathies.
Phillips EH et al.
Journal of Thrombosis and Haemostasis : Jth, 2016
Eculizumab in an anephric patient with atypical haemolytic uraemic syndrome and advanced vascular lesions.
Békássy ZD et al.
Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association, 2013
Access to the complement factor B scissile bond is facilitated by association of factor B with C3b protein.
Hourcade DE et al.
The Journal of Biological Chemistry, 2011
Lessons from functional and structural analyses of disease-associated genetic variants in the complement alternative pathway.
Rodríguez de Córdoba S et al.
Biochimica Et Biophysica Acta, 2011
Hyperfunctional C3 convertase leads to complement deposition on endothelial cells and contributes to atypical hemolytic uremic syndrome.
Roumenina LT et al.
Blood, 2009
Novel Variation in CFB Adult Onset Atypical Hemolytic Uremic Syndrome: A Case Report and Review.
Chhakchhuak M et al.
Indian Journal of Nephrology, 0