Complement Factor I Variants in Complement-Mediated Renal Diseases. [PDF]
Front Immunol, 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
Zhang Y +12 more
europepmc +2 more sources
Heme Interferes With Complement Factor I-Dependent Regulation by Enhancing Alternative Pathway Activation. [PDF]
Front Immunol, 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.
Gerogianni A +11 more
europepmc +2 more sources
Sequential Increase in Complement Factor I, iC3b, and Cells Expressing CD11b or CD14 in Cutaneous Vasculitis. [PDF]
Anal Cell Pathol (Amst), 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.
Rahkola D +4 more
europepmc +2 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 +2 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 +2 more sources
Functional expression of complement factor I following AAV-mediated gene delivery in the retina of mice and human cells. [PDF]
Gene Ther, 2021 Dry age-related macular degeneration (AMD) is characterised by loss of central vision and currently has no approved medical treatment. Dysregulation of the complement system is thought to play an important role in disease pathology and supplementation of
Dreismann AK +6 more
europepmc +2 more sources
A Candidate Prognostic Biomarker Complement Factor I Promotes Malignant Progression in Glioma. [PDF]
Front Cell Dev Biol, 2020 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.
Cai X +7 more
europepmc +2 more sources
Complement factor I deficiency: A potentially treatable cause of fulminant cerebral inflammation. [PDF]
Neurol Neuroimmunol Neuroinflamm, 2020 Objective To raise awareness of complement factor I (CFI) deficiency as a potentially treatable cause of severe cerebral inflammation. Methods Case report with neuroradiology, neuropathology, and functional data describing the mutation with review of ...
Altmann T +7 more
europepmc +2 more sources
Rare Genetic Variants in Complement Factor I Lead to Low FI Plasma Levels Resulting in Increased Risk of Age-Related Macular Degeneration. [PDF]
Invest Ophthalmol Vis Sci, 2020 Purpose Rare genetic variants in complement factor I (CFI) that cause low systemic levels of the protein (FI) have been reported as a strong risk factor for advanced age-related macular degeneration (AMD).
Hallam TM +8 more
europepmc +2 more sources
Complement Factor I Mutation May Contribute to Development of Thrombotic Microangiopathy in Lupus Nephritis. [PDF]
Front Med (Lausanne), 2020 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.
Tseng MH +8 more
europepmc +2 more sources