[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 …
Alternative complement pathway assessment in patients with atypical HUS
LT Roumenina, C Loirat, MA Dragon-Durey… - Journal of …, 2011 - Elsevier
The atypical Hemolytic Uremic Syndrome (aHUS) is a rare thrombotic microangiopathy
leading to end stage renal disease in approximately 60% of patients. Over the last decade, a …
leading to end stage renal disease in approximately 60% of patients. Over the last decade, a …
Complement gene variants determine the risk of immunoglobulin-associated MPGN and C3 glomerulopathy and predict long-term renal outcome
Background Membranoproliferative glomerulonephritis (MPGN) is an uncommon cause of
chronic nephropathy recently reclassified into immunoglobulin-associated MPGN (Ig-MPGN) …
chronic nephropathy recently reclassified into immunoglobulin-associated MPGN (Ig-MPGN) …
Regulator-dependent mechanisms of C3b processing by factor I allow differentiation of immune responses
The complement system labels microbes and host debris for clearance. Degradation of
surface-bound C3b is pivotal to direct immune responses and protect host cells. How the …
surface-bound C3b is pivotal to direct immune responses and protect host cells. How the …
Structural basis for complement factor I control and its disease-associated sequence polymorphisms
The complement system is a key component of innate and adaptive immune responses.
Complement regulation is critical for prevention and control of disease. We have determined …
Complement regulation is critical for prevention and control of disease. We have determined …
Heme interferes with complement factor I-dependent regulation by enhancing alternative pathway activation
Hemolysis, as a result of disease or exposure to biomaterials, is characterized by excess
amounts of cell-free heme intravascularly and consumption of the protective heme …
amounts of cell-free heme intravascularly and consumption of the protective heme …
Effect of rare coding variants in the CFI gene on Factor I expression levels
S de Jong, EB Volokhina, A de Breuk… - Human Molecular …, 2020 - academic.oup.com
Factor I (FI) is one of the main inhibitors of complement activity, and numerous rare coding
variants have been reported in patients with age-related macular degeneration, atypical …
variants have been reported in patients with age-related macular degeneration, atypical …
Functional analysis of variants in complement factor I identified in age-related macular degeneration and atypical hemolytic uremic syndrome
S de Jong, A de Breuk, B Bakker, S Katti… - Frontiers in …, 2022 - frontiersin.org
Complement factor I (FI) is a central inhibitor of the complement system, and impaired FI
function increases complement activation, contributing to diseases such as age-related …
function increases complement activation, contributing to diseases such as age-related …
[HTML][HTML] Complement factor I: Regulatory nexus, driver of immunopathology, and therapeutic
TM Hallam, SJ Sharp, A Andreadi, D Kavanagh - Immunobiology, 2023 - Elsevier
Complement factor I (FI) is the nexus for classical, lectin and alternative pathway
complement regulation. FI is an 88kDa plasma protein that circulates in an inactive …
complement regulation. FI is an 88kDa plasma protein that circulates in an inactive …
Classical and non-classical presentations of complement factor I deficiency: two contrasting cases diagnosed via genetic and genomic methods
AM Shields, AT Pagnamenta, AJ Pollard… - Frontiers in …, 2019 - frontiersin.org
Deficiency of complement factor I is a rare immunodeficiency that typically presents with
increased susceptibility to encapsulated bacterial infections. However, non-infectious …
increased susceptibility to encapsulated bacterial infections. However, non-infectious …