Results 11 to 20 of about 1,866,008 (218)

Cutaneous Vasculitis and Recurrent Infection Caused by Deficiency in Complement Factor I. [PDF]

Front Immunol, 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 ...
Nanthapisal S, Eleftheriou D, Gilmour K, Leone V, Ramnath R, Omoyinmi E, Hong Y, Klein N, Brogan PA.   +8 more
europepmc   +4 more sources

Classical and Non-classical Presentations of Complement Factor I Deficiency: Two Contrasting Cases Diagnosed via Genetic and Genomic Methods. [PDF]

Front Immunol, 2019
Deficiency of complement factor I is a rare immunodeficiency that typically presents with increased susceptibility to encapsulated bacterial infections.
Shields AM, Pagnamenta AT, Pollard AJ, OxClinWGS, Taylor JC, Allroggen H, Patel SY.   +6 more
europepmc   +4 more sources

Functional Analysis of Rare Genetic Variants in Complement Factor I (CFI) using a Serum-Based Assay in Advanced Age-related Macular Degeneration. [PDF]

Transl Vis Sci Technol, 2020
Purpose Factor I (FI) is a serine protease regulator of the complement system. Genetic variants in CFI are associated with advanced age-related macular degeneration (AAMD). However, the clinical and functional impact of these variants is unknown.
Java A, Baciu P, Widjajahakim R, Sung YJ, Yang J, Kavanagh D, Atkinson J, Seddon J.   +7 more
europepmc   +2 more sources

Serum complement factor I is associated with disease activity of systemic lupus erythematosus. [PDF]

Oncotarget, 2018
Although aberrant complement activation is involved in the pathogenesis of systemic lupus erythematosus (SLE), the role of complement regulatory proteins in disease activity of SLE remains limited.
Tseng MH, Lin SH, Wu CY, Chien HP, Yang HY, Chen YC, Chou YC, Huang JL.   +7 more
europepmc   +2 more sources

Plasma Levels of Complement Factor I and C4b Peptides Are Associated with HIV Suppression. [PDF]

ACS Infect Dis, 2017
Individuals who exhibit long-term HIV suppression and CD4 T-cell preservation without antiretroviral therapy are of great interest for HIV research. There is currently no robust method for rapid identification of these “HIV controller” subjects; however,
Wu B, Ouyang Z, Lyon CJ, Zhang W, Clift T, Bone CR, Li B, Zhao Z, Kimata JT, Yu XG, Hu Y.   +10 more
europepmc   +2 more sources

Functional evaluation of complement factor I variants by immunoassays and SDS-PAGE. [PDF]

Front Immunol, 2023
Factor I (FI) is an essential regulator of the complement system. Together with co-factors, FI degrades C3b, which inhibits further complement activation.
Gerogianni A, Baas LM, Sjöström DJ, van de Kar NCAJ, Pullen M, van de Peppel SJ, Nilsson PH, van den Heuvel LP.   +7 more
europepmc   +2 more sources

Complement factor I deficiency: a not so rare immune defect. Characterization of new mutations and the first large gene deletion

Orphanet Journal of Rare Diseases, 2012
BackgroundDystrophin is a large essential protein of skeletal and heart muscle. It is a filamentous scaffolding protein with numerous binding domains. Mutations in the DMD gene, which encodes dystrophin, mostly result in the deletion of one or several ...
María Alba-Domínguez, A. López-Lera, S. Garrido, P. Nozal, Ignacio González-Granado, Josefa Melero, P. Soler-Palacín, C. Cámara, M. López-Trascasa   +8 more
semanticscholar   +3 more sources

Functional evaluation of rare variants in complement factor I using a minigene assay. [PDF]

Front Immunol
The regulatory serine protease, complement factor I (FI), in conjunction with one of its cofactors (FH, C4BP, MCP, or CR1), plays an essential role in controlling complement activity through inactivation of C3b and C4b.
Donelson CJH, Ghiringhelli Borsa N, Taylor AO, Smith RJH, Zhang Y.   +4 more
europepmc   +2 more sources

A novel missense mutation in complement factor I predisposes patients to atypical hemolytic uremic syndrome: a case report. [PDF]

J Med Case Rep, 2022
Background Atypical hemolytic uremic syndrome, also called the nondiarrheal form of hemolytic uremic syndrome, is a rare disease characterized by the triad of thrombocytopenia, Coomb’s test-negative microangiopathic hemolytic anemia, and acute renal ...
Wei X, Li J, Zhan X, Tu L, Huang H, Wang Y.   +5 more
europepmc   +2 more sources

Local expression of complement factor I in breast cancer cells correlates with poor survival and recurrence. [PDF]

Cancer Immunol Immunother, 2015
Okroj M, Holmquist E, Nilsson E, Anagnostaki L, Jirström K, Blom AM.   +5 more
europepmc   +2 more sources