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anti-Mouse (Murine) Complement Factor I Anticorps:
anti-Human Complement Factor I Anticorps:
anti-Rat (Rattus) Complement Factor I Anticorps:
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Human Monoclonal Complement Factor I Primary Antibody pour Func, IHC (fro) - ABIN2473071
Ernberg: Studies on Epstein-Barr virus-associated antigens. dans Annals of the New York Academy of Sciences 1975
Show all 2 Pubmed References
Factor I-mediated generation of activated C3 fragments in the circulation is a critical determinant for the development of MPGN2 associated with factor H deficiency.
CFI deficiency is thus now added to the growing list of monogenic causes of vasculitis and should always be considered in vasculitis patients found to have persistently low levels of C3 with normal C4.
A high occurrence of Ile357Met mutation and association of simultaneous genetic abnormalities of CFI characterized our Tunisian Atypical Hemolytic and Uremic syndrome patients.
CFI rs141853578 (G119R) is a risk factor for developing advanced type AMD. This study also suggests that the frequency of G119R polymorphism in our population is not as rare as reported from other populations.
This study has revealed a significant genetic role for CFI-rs13104777 in acute anterior uveitis. This influence may be dependent on human leukocyte antigen (HLA)-B27 and disease laterality.
An extremely rare, heterozygous mutation in the gene encoding CFI likely affecting splicing was associated for the first time with atypical hemolytic uremic syndrome.
this study illustrates the importance of early versus late diagnosis of CFI deficiency
This finding although rare does suggest that screening for chromosomal rearrangements affecting CFI should be undertaken in all aHUS patients particularly if the factor I level is unexplainably low.
Factor I binds C3b-Factor H between Factor H domains 2 and 3 and a reoriented C3b C-terminal domain and docks onto the first scissile bond, while stabilizing its catalytic domain for proteolytic activity.
Taken together, our data argue that multiple rare and ultra-rare alleles in CFI contribute to AMD pathogenesis; they improve the precision of the assessment of the contribution of CFI to AMD
Case Report: thrombotic microangiopathy with mutations in complement factor I and thrombomodulin.
Our results indicate that CFI polymorphisms are not significantly associated with VKH syndrome.
Patients with advanced atrophic AMD carried these rare variants more frequently than patients with neovascular AMD (11 of 93 [11.8%] vs 40 of 835 [4.8%]; P = .04).
Low FI levels are strongly associated with rare CFI variants and age-related macular degeneration.
A missense variant (p.V412M) in CFI was discovered in two Tunisian Jewish families with early-onset age-related macular degeneration.
Regulatory components of the alternative complement pathway in endothelial cell cytoplasm, factor H and factor I, are not packaged in Weibel-Palade bodies.
In this study, the odds of AMD were highest in those with deficient vitamin D status and 2 risk alleles for the CFH and CFI genotypes, suggesting a synergistic effect between vitamin D status and complement cascade protein function.
iC3b level, a proteolytically inactive form of C3b, was lower in HCV infected patient sera, reflecting impairment of both C3 convertase and Factor I activity.
association between rs10033900 and age-related macular degeneration risk in Han Chinese population
The mutations in the regulators CFH, CFI and MCP involve loss-of-function, whereas those for C3 involve gain-of-function.
The CFI p.Gly119Arg mutation was identified in 7/521 age-related macular degeneration cases compared to 1/627 age-matched controls; this mutation confers a high risk of disease.
This gene encodes a serine proteinase that is essential for regulating the complement cascade. The encoded preproprotein is cleaved to produce both heavy and light chains, which are linked by disulfide bonds to form a heterodimeric glycoprotein. This heterodimer can cleave and inactivate the complement components C4b and C3b, and it prevents the assembly of the C3 and C5 convertase enzymes. Defects in this gene cause complement factor I deficiency, an autosomal recessive disease associated with a susceptibility to pyogenic infections. Mutations in this gene have been associated with a predisposition to atypical hemolytic uraemic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immmune deposits is another condition associated with mutation of this gene.
complement factor I
, I factor (complement)
, complement factor 1
, C3B/C4B inactivator
, Konglutinogen-activating factor
, complement component I
, complement control protein factor I
, complement factor I heavy chain
, light chain of factor I
, complement component 1