Complement Factor I Variants in Complement-Mediated Renal Diseases [PDF]
Frontiers in Immunology, 2022 C3 glomerulopathy (C3G) and atypical hemolytic uremic syndrome (aHUS) are two rare diseases caused by dysregulated activity of the alternative pathway of complement secondary to the presence of genetic and/or acquired factors. Complement factor I (FI) is
Yuzhou Zhang +12 more
doaj +7 more sources
Cutaneous Vasculitis and Recurrent Infection Caused by Deficiency in Complement Factor I [PDF]
Frontiers in Immunology, 2018 Cutaneous leukocytoclastic vasculitis arises from immune complex deposition and dysregulated complement activation in small blood vessels. There are many causes, including dysregulated host response to infection, drug reactions, and various autoimmune ...
Sira Nanthapisal +9 more
doaj +7 more sources
Complement Factor I Mutation May Contribute to Development of Thrombotic Microangiopathy in Lupus Nephritis [PDF]
Frontiers in Medicine, 2021 Objective: Renal thrombotic microangiopathy (TMA) is associated with complement overactivation and poor outcome in patients with lupus nephritis (LN). The role of genetic makeup of complement system in these patients remains to be elucidated.Methods: The
Min-Hua Tseng +14 more
doaj +5 more sources
Prevalence and phenotype associations of complement factor I mutations in geographic atrophy. [PDF]
Hum Mutat, 2021 Rare variants in the complement factor I (CFI) gene, associated with low serum factor I (FI) levels, are strong risk factors for developing the advanced stages of age‐related macular degeneration (AMD).
Khan AH +19 more
europepmc +9 more sources
Classical and Non-classical Presentations of Complement Factor I Deficiency: Two Contrasting Cases Diagnosed via Genetic and Genomic Methods [PDF]
Frontiers in Immunology, 2019 Deficiency of complement factor I is a rare immunodeficiency that typically presents with increased susceptibility to encapsulated bacterial infections.
Adrian M. Shields +22 more
doaj +5 more sources
Heme Interferes With Complement Factor I-Dependent Regulation by Enhancing Alternative Pathway Activation [PDF]
Frontiers in Immunology, 2022 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-scavenger proteins in plasma.
Alexandra Gerogianni +20 more
doaj +3 more sources
A Candidate Prognostic Biomarker Complement Factor I Promotes Malignant Progression in Glioma [PDF]
Frontiers in Cell and Developmental Biology, 2021 Objectives: Glioma is the most common and aggressive type of primary central nervous system (CNS) tumor in adults and is associated with substantial mortality rates.
Xiaomin Cai +7 more
doaj +3 more sources
Functional evaluation of complement factor I variants by immunoassays and SDS-PAGE [PDF]
Frontiers in Immunology, 2023 Factor I (FI) is an essential regulator of the complement system. Together with co-factors, FI degrades C3b, which inhibits further complement activation.
Alexandra Gerogianni +13 more
doaj +6 more sources
Sequential Increase in Complement Factor I, iC3b, and Cells Expressing CD11b or CD14 in Cutaneous Vasculitis [PDF]
Analytical Cellular Pathology, 2022 Mast cells contribute to the pathogenesis of cutaneous vasculitis through complement C3 that is cleaved to C3b and then to iC3b by complement factor I. The receptor of iC3b, CD11b, is expressed on neutrophils and monocytes and CD14 on monocytes.
Dina Rahkola +4 more
doaj +3 more sources
Functional Analysis of Variants in Complement Factor I Identified in Age-Related Macular Degeneration and Atypical Hemolytic Uremic Syndrome. [PDF]
Front Immunol, 2021 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 macular degeneration (AMD) and atypical hemolytic uremic syndrome (aHUS ...
de Jong S +9 more
europepmc +4 more sources