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complement component C3 + H2O
?
structural but not functional roles for the three N-linked oligosaccharide chains indicated, N-linked glycan pool composition of the heavy and light chains indicated
-
-
?
complement component C3b + H2O
complement component C3c + ?
complement component C4b + H2O
complement component C4c + C4d
benzoyl-Arg-4-methylcoumaryl-7-amide + H2O
benzoyl-Arg + 7-amino-4-methylcoumarin
-
low activity
-
?
benzyloxycarbonyl-Gly-Gly-Arg-4-methylcoumaryl-7-amide + H2O
benzyloxycarbonyl-Gly-Gly-Arg + 7-amino-4-methylcoumarin
-
low activity
-
?
benzyloxycarbonyl-Gly-Pro-Arg-4-methylcoumaryl-7-amide + H2O
benzyloxycarbonyl-Gly-Pro-Arg + 7-amino-4-methylcoumarin
-
-
-
?
benzyloxycarbonyl-Leu-Leu-Arg-4-methylcoumaryl-7-amide + H2O
benzyloxycarbonyl-Leu-Leu-Arg + 7-amino-4-methylcoumarin
-
-
-
?
benzyloxycarbonyl-Phe-Arg-4-methylcoumaryl-7-amide + H2O
benzyloxycarbonyl-Phe-Arg + 7-amino-4-methylcoumarin
-
low activity
-
?
Boc-Asp(benzyl)-Pro-Arg-4-methylcoumarin 7-amide + H2O
?
-
-
-
-
?
complement component C3(NH3) + H2O
?
-
cleaved by SP domain-form
-
-
?
complement component C3b + H2O
?
complement component C3b + H2O
complement component C3c + ?
complement component C3b + H2O
complement component iC3b
-
-
a major opsonin
-
?
complement component C3b + H2O
complement component iC3b + ?
complement component C3b + H2O
inactivated C3b + ?
complement component C3bi + H2O
?
-
the breakdown of human erythrocyte-bound C3bi molecules in serum or plasma is mediated only by factor I
-
-
?
complement component C3bi + H2O
complement component C3c + ?
-
complement component C3bi bound to human erythrocytes is rapidly cleaved, unlike complement component C3bi bound to other species
-
?
complement component C4b + H2O
?
complement component C4b + H2O
complement component C4c + C4d
complement factor C3b + H2O
?
-
-
-
-
?
complement factor C4b + H2O
?
-
-
-
-
?
FGR-7-amido-4-methylcoumarin + H2O
FGR + 7-amino-4-methylcoumarin
-
-
-
-
?
methylsulfonyl-D-Phe-Gly-Arg-4-methylcoumaryl-7-amide + H2O
methylsulfonyl-D-Phe-Gly-Arg + 7-amino-4-methylcoumarin
-
-
-
?
N-alpha-tert-butyloxycarbonyl-Val-Leu-Lys-4-methylcoumaryl-7-amide + H2O
N-alpha-tert-butyloxycarbonyl-Val-Pro-Lys + 7-amino-4-methylcoumarin
-
low activity
-
?
N-alpha-tert-butyloxycarbonyl-Val-Pro-Arg-4-methylcoumaryl-7-amide + H2O
N-alpha-tert-butyloxycarbonyl-Val-Pro-Arg + 7-amino-4-methylcoumarin
-
-
-
?
Phe-Gly-Arg-4-methylcoumaryl-7-amide + H2O
Phe-Gly-Arg + 7-amino-4-methylcoumarin
-
-
-
?
Pro-Phe-Arg-4-methylcoumaryl-7-amide + H2O
Pro-Phe-Arg + 7-amino-4-methylcoumarin
-
low activity
-
?
tert-butyloxycarbonyl-Asp(benzyl ester)-Pro-Arg-4-methylcoumaryl-7-amide + H2O
tert-butyloxycarbonyl-Asp(benzyl ester)-Pro-Arg + 7-amino-4-methylcoumarin
-
-
-
?
tert-butyloxycarbonyl-Asp(benzyl)-Pro-Arg-7-amido-4-methylcoumarin + H2O
?
-
-
-
-
?
tert-butyloxycarbonyl-Ile-Glu-Gly-Arg-4-methylcoumaryl-7-amide + H2O
tert-butyloxycarbonyl-Ile-Glu-Gly-Arg + 7-amino-4-methylcoumarin
-
low activity
-
?
tert-butyloxycarbonyl-Phe-Ser-Arg-4-methylcoumaryl-7-amide + H2O
tert-butyloxycarbonyl-Phe-Ser-Arg + 7-amino-4-methylcoumarin
-
low activity
-
?
additional information
?
-
complement component C3b + H2O
complement component C3c + ?
-
-
-
?
complement component C3b + H2O
complement component C3c + ?
mutation of complement factor I associated to atypical hemolytic uremic syndrome analyzed, activity observed in mutant protein comparable to wild-type
-
-
?
complement component C3b + H2O
complement component C3c + ?
complement factor I mutants predisposing to atypical hemolytic uremic syndrome analyzed, cofactor function of complement components C3b and Cb4 tested
-
-
?
complement component C3b + H2O
complement component C3c + ?
complement factor I production of human keratinocytes analyzed, with and without stimulation by IFN-gamma, IL-1alpha, IL-6, TNF-alpha, TGF-beta1
-
-
?
complement component C3b + H2O
complement component C3c + ?
production of complement factor I in non-small cell lung cancer cell lines analyzed
-
-
?
complement component C4b + H2O
complement component C4c + C4d
-
-
-
?
complement component C4b + H2O
complement component C4c + C4d
-
-
-
-
?
complement component C4b + H2O
complement component C4c + C4d
-
-
-
?
complement component C3b + H2O
?
-
-
-
-
?
complement component C3b + H2O
?
-
cleavage at five different sites in the alpha-chain, generating complement component C3d.g-like fragments with three different N-terminal and two different C-terminal ends
-
-
?
complement component C3b + H2O
?
-
degradation in presence of C4b binding protein and factor H
-
-
?
complement component C3b + H2O
complement component C3c + ?
-
-
-
-
?
complement component C3b + H2O
complement component C3c + ?
-
-
-
?
complement component C3b + H2O
complement component C3c + ?
-
-
-
-
?
complement component C3b + H2O
complement component C3c + ?
-
phosphorylation of complement component C3b inhibits the cleavage of the alpha'-chain of complement component C3b
-
-
?
complement component C3b + H2O
complement component C3c + ?
-
cleavage at five different sites in the alpha-chain, generating complement component C3d.g-like fragments with three different N-terminal and two different C-terminal ends
-
-
?
complement component C3b + H2O
complement component iC3b + ?
-
cleavage does not require presence of factor H
-
?
complement component C3b + H2O
complement component iC3b + ?
-
the complement C3 fragments C3b and iC3b appear on the surface of several virulent Staphylococcus aureus strains of capsule polysaccharide type 5 and 8. Factor I mediates the cleavage of C3b to iC3b on the surface of Staphylococcus aureus and appears to be able to function without the serum cofactor, factor H
-
?
complement component C3b + H2O
inactivated C3b + ?
-
-
-
-
?
complement component C3b + H2O
inactivated C3b + ?
-
factor I mediates cleavage of C3b between Arg1298 and Ser1299
-
-
?
complement component C4b + H2O
?
-
-
-
-
?
complement component C4b + H2O
?
-
degradation in presence of C4b binding protein and factor H
-
-
?
complement component C4b + H2O
complement component C4c + C4d
-
-
-
-
?
complement component C4b + H2O
complement component C4c + C4d
-
a nicked form of complement component C4b, complement component C4b', consisting of four disulfide-linked polypeptide chains, is formed as an intermediate product before cleavage of complement component C4b into complement component C4c and complement component C4d
-
?
additional information
?
-
complement factor I production analyzed by RT-PCR
-
-
?
additional information
?
-
complement factor I production analyzed, no mRNA expression detected
-
-
?
additional information
?
-
production of complement factor I analyzed, no mRNA expression detected
-
-
?
additional information
?
-
human factor I has little species-specificity like pig factor I, according to the relatively high homology of the AA sequences in the serine protease region, in comparison with those of membrane complement regulatory proteins
-
-
?
additional information
?
-
-
human factor I has little species-specificity like pig factor I, according to the relatively high homology of the AA sequences in the serine protease region, in comparison with those of membrane complement regulatory proteins
-
-
?
additional information
?
-
-
no cleavage of H-Gly-Arg-7-amido-4-methylcoumarin, MeOSuc-Ala-Ala-Pro-Val-7-amido-4-methylcoumarin or N-alpha-tert-butyloxycarbonyl-leu-Gly-Arg-7-amido-4-methylcoumarin
-
?
additional information
?
-
-
Staphylococcus aureus expressing clumping factor A (ClfA) (P336A Y338S) is more susceptible to complement-mediated phagocytosis than a ClfA-null mutant or the wild type. Unlike clumping factor A, the mutant ClfA(P336A Y338S) does not enhance factor I cleavage of C3b to iC3b and inhibits the cofactor function of factor H. Fibrinogen enhances factor I binding to clumping factor A and the Staphylococcus aureus surface
-
-
?
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2-mercaptoethanol
-
10 mM, strong
4-(2-aminoethyl)benzenesulfonyl fluoride
-
0.25 mM, inhibits SP domain form and fI
amyloid beta
-
binds to complement factor I which inhibits the ability of factor I to cleave C3b to inactivated C3b. Addition of factor I restores inactivated C3b production in amyloid beta-treated RPE cells. Preincubation of factor I with amyloid beta in the presence of factor H abolishes the ability of factor I to cleave FGR-7-amino-4-methylcoumarin
-
benzamidine
-
20 mM, 82% inhibition of amidolytic activity
benzenesulfonyl fluorides
-
inhibits amidolytic activity
benzyloxycarbonyl-D-Phe-Pro-methoxypropylboroglycinepinanediol ester
Cr2+
-
inhibition of proteolytic and amidolytic activity, 54% inhibition of amidolytic activity at 1 mM, 43% inhibition of proteolytic activity at 0.1 mM
Cu2+
-
23% inhibition of amidolytic activity at 1 mM
diisopropylfluorophosphate
-
-
dithiothreitol
-
1 mM, strong
epsilon-aminocaproic acid
-
20 mM, 10% inhibition of amidolytic activity
factor H
-
inhibits SP domain form
-
Fe3+
-
inhibition of proteolytic and amidolytic activity, 59% inhibition of amidolytic activity at 1 mM, 23% inhibition of proteolytic activity at 0.1 mM
Hg2+
-
26% inhibition of amidolytic activity at 1 mM
Lima bean trypsin inhibitor
-
0.05 mM, 20% inhibition of amidolytic activity
-
NEM
-
1 mM, partial inhibition
Pefabloc TH
-
0.25 mM, 58% inhibition of amidolytic activity
Pefabloc-SC
-
0.00025 mM, complete inhibition of amidolytic activity
Pefabloc-Xa
-
0.00025 mM, 93% inhibition of amidolytic activity
PMSF
-
1 mM, 42% inhibition of amidolytic activity
Soybean trypsin inhibitor
-
0.05 mM, 37% inhibition of amidolytic activity
-
Zn2+
-
0.1 mM, 59% inhibition of proteolytic activity
antipain
-
0.1 mM, 80% inhibition of amidolytic activity
antipain
-
0.1 mM, inhibits SP domain form and fI
Aprotinin
-
0.0005 mM, 23% inhibition of proteolytic activity. 0.0005 mM, 37% inhibition of amidolytic activity
Aprotinin
-
0.005 mM, inhibits SP domain form and fI
benzyloxycarbonyl-D-Phe-Pro-methoxypropylboroglycinepinanediol ester
-
0.05 mM, 82% inhibition
benzyloxycarbonyl-D-Phe-Pro-methoxypropylboroglycinepinanediol ester
-
inhibits amidolytic activity
leupeptin
-
0.01 mM, 24% inhibition of amidolytic activity
leupeptin
-
0.01 mM, 29% inhibition of proteolytic activity
leupeptin
-
0.01 mM, inhibits SP domain form and fI
suramin
-
weak inhibition
suramin
-
1 mM, 90% inhibition of proteolytic activity. 1 mM, 87% inhibition of amidolytic activity
suramin
-
1 mM, inhibits fI
additional information
-
phosphorylation of C3b inhibits the cleavage of the alpha'-chain of C3b
-
additional information
-
no inhibition by 0.1 mM 1,10-phenanthroline, 0.001 mM pepstatin A, 0.1 mM chymostatin, 0.0002 mM, C1 inhibitor and 0.1 mM bestatin
-
additional information
-
factor I is in a proteolytically inactive form, it circulates in a zymogen-like state despite being fully processed to the mature sequence. This inactive form is maintained by the noncatalytic heavy-chain allosterically modulating activity of the light chain. Once the ternary complex of factor I, a cofactor and a substrate is formed, the allosteric inhibition is released, and factor I is oriented for cleavage
-
additional information
-
the enzyme does not have any known physiological inhibitor, although synthetic inhibitors, such as suramin, are able to weakly inhibit it
-
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Alzheimer Disease
A monoclonal antibody targeting amyloid ? (A?) restores complement factor I bioactivity: Potential implications in age-related macular degeneration and Alzheimer's disease.
Angioedema
The role of complement in urticaria and angioedema.
Arthritis, Rheumatoid
Alternative pathway complement activation in rheumatoid arthritis.
Arthritis, Rheumatoid
C3b inactivator in the rheumatic diseases. Measurement by radial immunodiffusion and by inhibition of formation of properdin pathway C3 convertase.
Arthritis, Rheumatoid
Increased biosynthesis of complement components by cultured monocytes, synovial fluid macrophages and skynovial membrane cells from patients with rheumatoid arthritis.
Atypical Hemolytic Uremic Syndrome
A Newly Identified Mutation in the Complement Factor I Gene Not Associated With Early Post-transplant Recurrence of Atypical Hemolytic-Uremic Syndrome: A Case Report.
Atypical Hemolytic Uremic Syndrome
An extremely rare splice site mutation in the gene encoding complement factor I in a patient with atypical hemolytic uremic syndrome.
Atypical Hemolytic Uremic Syndrome
Atypical hemolytic-uremic syndrome due to complement factor I mutation.
Atypical Hemolytic Uremic Syndrome
Eculizumab for the treatment of an atypical hemolytic uremic syndrome with mutations in complement factor I and C3.
Atypical Hemolytic Uremic Syndrome
Excessive activation of the complement system in atypical hemolytic uremic syndrome: is it ready for prime time?
Atypical Hemolytic Uremic Syndrome
Mutations in complement factor I as found in atypical hemolytic uremic syndrome lead either to altered secretion or altered function of factor I.
Atypical Hemolytic Uremic Syndrome
Mutations in complement factor I predispose to development of atypical hemolytic uremic syndrome.
Atypical Hemolytic Uremic Syndrome
Simultaneous acute pancreatitis and angioedema associated with angiotensin-converting enzyme inhibitor.
Bacterial Infections
Classical and Non-classical Presentations of Complement Factor I Deficiency: Two Contrasting Cases Diagnosed via Genetic and Genomic Methods.
Bacterial Infections
[Hereditary complement deficiencies]
Breast Neoplasms
Local expression of complement factor I in breast cancer cells correlates with poor survival and recurrence.
Bronchiectasis
C3b inactivator deficiency with immune complex manifestations.
Carcinoma
Complement factor I promotes progression of cutaneous squamous cell carcinoma.
Carcinoma, Hepatocellular
Biosynthesis and postsynthetic processing of human C3b/C4b inactivator (factor I) in three hepatoma cell lines.
Carcinoma, Non-Small-Cell Lung
Non-small cell lung cancer cells produce a functional set of complement factor I and its soluble cofactors.
Carcinoma, Non-Small-Cell Lung
Tag SNPs of CFI contributed to the susceptibility for non-small cell lung cancer in Chinese population.
Carcinoma, Squamous Cell
Complement factor I promotes progression of cutaneous squamous cell carcinoma.
Carcinoma, Squamous Cell
Complement factor I upregulates expression of matrix metalloproteinase-13 and -2 and promotes invasion of cutaneous squamous carcinoma cells.
Choroidal Neovascularization
Evaluation of pigment epithelium-derived factor and complement factor I polymorphisms as a cause of choroidal neovascularization in highly myopic eyes.
Colitis, Ulcerative
Complement inhibitors and immunoconglutinins in ulcerative colitis and Crohn's disease.
complement factor i deficiency
A family with complement factor I deficiency.
complement factor i deficiency
C3b inactivator deficiency with immune complex manifestations.
complement factor i deficiency
Classical and Non-classical Presentations of Complement Factor I Deficiency: Two Contrasting Cases Diagnosed via Genetic and Genomic Methods.
complement factor i deficiency
Complement factor I deficiency associated with recurrent infections, vasculitis and immune complex glomerulonephritis.
complement factor i deficiency
Complement factor I deficiency associated with recurrent meningitis coinciding with menstruation.
complement factor i deficiency
Complement factor I deficiency in a 3-year-old boy with glomerulonephritis.
complement factor i deficiency
Complement factor I deficiency in a family with recurrent infections.
complement factor i deficiency
Complement factor I deficiency with recurrent aseptic meningitis.
complement factor i deficiency
Complement factor I deficiency: a not so rare immune defect. characterization of new mutations and the first large gene deletion.
complement factor i deficiency
Complement factor I deficiency: A potentially treatable cause of fulminant cerebral inflammation.
complement factor i deficiency
Complement-activating ability of leucocytes from patients with complement factor I deficiency.
complement factor i deficiency
Control of serum C3 levels by beta 1H and C3b inactivator.
complement factor i deficiency
Early Versus Late Diagnosis of Complement Factor I Deficiency: Clinical Consequences Illustrated in Two Families with Novel Homozygous CFI Mutations.
complement factor i deficiency
Genetic, molecular and functional analyses of complement factor I deficiency.
complement factor i deficiency
Glomerulonephritis in a patient with complement factor I deficiency.
complement factor i deficiency
Hereditary complement factor I deficiency.
complement factor i deficiency
Molecular basis of complement factor I deficiency in Tunisian atypical hemolytic and uremic syndrome Patients.
complement factor i deficiency
Molecular characterization of Complement Factor I deficiency in two Spanish families.
complement factor i deficiency
Molecular characterization of two novel cases of complete complement inhibitor Factor I deficiency.
complement factor i deficiency
Novel CFI mutation in a patient with leukocytoclastic vasculitis may redefine the clinical spectrum of Complement Factor I deficiency.
complement factor i deficiency
Plasma C3d levels as a diagnostic marker for complete complement factor I deficiency.
complement factor i deficiency
Primary Pneumococcal Peritonitis can be the first presentation of a familial complement factor I deficiency.
complement factor i deficiency
Recurrent infections in partial complement factor I deficiency: evaluation of three generations of a Brazilian family.
complement factor i deficiency
Restoration by purified C3b inactivator of complement-mediated function in vivo in a patient with C3b inactivator deficiency.
complement factor i deficiency
Simultaneous acute pancreatitis and angioedema associated with angiotensin-converting enzyme inhibitor.
complement factor i deficiency
The molecular basis of hereditary complement factor I deficiency.
complement factor i deficiency
[Complement factor I deficiency revealed by repeated systemic Streptococcus pneumoniae infection]
Crohn Disease
Complement inhibitors and immunoconglutinins in ulcerative colitis and Crohn's disease.
Diabetes Mellitus, Type 1
Complements in diabetes mellitus: activation of complement system evidenced by C3d elevation in IDDM.
Diabetes Mellitus, Type 2
Complements in diabetes mellitus: activation of complement system evidenced by C3d elevation in IDDM.
Diabetes Mellitus, Type 2
Complements in non-insulin-dependent diabetes mellitus with complications.
Geographic Atrophy
Association Between Growth of Geographic Atrophy and the Complement Factor I Locus.
Geographic Atrophy
Prevalence and phenotype associations of complement factor I mutations in geographic atrophy.
Geographic Atrophy
Targeting factor D of the alternative complement pathway reduces geographic atrophy progression secondary to age-related macular degeneration.
Glioma
A Candidate Prognostic Biomarker Complement Factor I Promotes Malignant Progression in Glioma.
Glomerulonephritis
C3 glomerulonephritis and thrombotic microangiopathy of renal allograft after pulmonary infection in a male with concomitant two complement factor I gene variations: a case report.
Glomerulonephritis
Complement factor I deficiency associated with recurrent infections, vasculitis and immune complex glomerulonephritis.
Glomerulonephritis
Complement factor I deficiency in a 3-year-old boy with glomerulonephritis.
Glomerulonephritis
Correlations between serum factor B and C3b inactivator levels in normal subjects and in patients with infections, nephrosis and hypocomplementaemic glomerulonephritis.
Glomerulonephritis
Evidence for glomerular modulation of complement activation.
Glomerulonephritis
Glomerulonephritis in a patient with complement factor I deficiency.
Glomerulonephritis
Modulation of the properdin amplification loop in membranoproliferative and other forms of glomerulonephritis.
Glomerulonephritis, Membranoproliferative
Control of complement activation in membranous and membranoproliferative glomerulonephritis.
Glomerulonephritis, Membranoproliferative
Control of serum C3 levels by beta 1H and C3b inactivator.
Hearing Loss, Noise-Induced
Transcriptome characterization by RNA-Seq reveals the involvement of the complement components in noise-traumatized rat cochleae.
HELLP Syndrome
The genetics of the alternative pathway of complement in the pathogenesis of HELLP syndrome.
Hemolytic-Uremic Syndrome
Characterization of mutations in complement factor I (CFI) associated with hemolytic uremic syndrome.
Hereditary Complement Deficiency Diseases
Genetic, molecular and functional analyses of complement factor I deficiency.
Infections
C3 glomerulonephritis and thrombotic microangiopathy of renal allograft after pulmonary infection in a male with concomitant two complement factor I gene variations: a case report.
Infections
Clinical laboratory standard capillary protein electrophoresis alerted of a low C3 state and lead to the identification of a Factor I deficiency due to a novel homozygous mutation.
Infections
Complement factor I deficiency associated with recurrent infections, vasculitis and immune complex glomerulonephritis.
Infections
Complement factor I deficiency in a family with recurrent infections.
Infections
Correlations between serum factor B and C3b inactivator levels in normal subjects and in patients with infections, nephrosis and hypocomplementaemic glomerulonephritis.
Infections
Cutaneous Vasculitis and Recurrent Infection Caused by Deficiency in Complement Factor I.
Infections
Genetic, molecular and functional analyses of complement factor I deficiency.
Infections
Glomerulonephritis in a patient with complement factor I deficiency.
Infections
Molecular characterization and expression of complement factor I in Pelteobagrus vachellii during Aeromonas hydrophila infection.
Infections
Recurrent infections in partial complement factor I deficiency: evaluation of three generations of a Brazilian family.
Infections
The effect of substance(s) associated with malarial parasites on C3b inactivator.
Infections
[Complement factor I deficiency revealed by repeated systemic Streptococcus pneumoniae infection]
Joint Diseases
C3b inactivator in the rheumatic diseases. Measurement by radial immunodiffusion and by inhibition of formation of properdin pathway C3 convertase.
Leukoencephalitis, Acute Hemorrhagic
Mutations of Complement Factor I and Potential Mechanisms of Neuroinflammation in Acute Hemorrhagic Leukoencephalitis.
Lung Neoplasms
Non-small cell lung cancer cells produce a functional set of complement factor I and its soluble cofactors.
Lung Neoplasms
Tag SNPs of CFI contributed to the susceptibility for non-small cell lung cancer in Chinese population.
Lupus Erythematosus, Systemic
C3b inactivator in the rheumatic diseases. Measurement by radial immunodiffusion and by inhibition of formation of properdin pathway C3 convertase.
Lupus Erythematosus, Systemic
Relative importance of C3b inactivator and beta 1H globulin in the modulation of the properdin amplification loop in systemic lupus erythematosus.
Lupus Erythematosus, Systemic
Serum complement factor I is associated with disease activity of systemic lupus erythematosus.
Lupus Nephritis
Complement Factor I Mutation May Contribute to Development of Thrombotic Microangiopathy in Lupus Nephritis.
Macular Degeneration
A monoclonal antibody targeting amyloid ? (A?) restores complement factor I bioactivity: Potential implications in age-related macular degeneration and Alzheimer's disease.
Macular Degeneration
Additional evidence to support the role of a common variant near the complement factor I gene in susceptibility to age-related macular degeneration.
Macular Degeneration
Age-related macular degeneration: Complement in action.
Macular Degeneration
Association between complement factor I gene polymorphisms and the risk of age-related macular degeneration: a Meta-analysis of literature.
Macular Degeneration
Association of polymorphisms of complement factor I rs141853578 (G119R) with age-related macular degeneration in Iranian population.
Macular Degeneration
Common variant rs10033900 near the complement factor I gene is associated with age-related macular degeneration risk in Han Chinese population.
Macular Degeneration
Complement factor I and age-related macular degeneration.
Macular Degeneration
Complement Factor I Gene Polymorphism in a Turkish Age-Related Macular Degeneration Population.
Macular Degeneration
Complement factor I polymorphism is not associated with neovascular age-related macular degeneration and polypoidal choroidal vasculopathy in a chinese population.
Macular Degeneration
Functional Analysis of Rare Genetic Variants in Complement Factor I (CFI) using a Serum-Based Assay in Advanced Age-related Macular Degeneration.
Macular Degeneration
Interactions among different genetic loci in age-related macular degeneration.
Macular Degeneration
No evidence of association between complement factor I genetic variant rs10033900 and age-related macular degeneration.
Macular Degeneration
Prevalence and phenotype associations of complement factor I mutations in geographic atrophy.
Macular Degeneration
Rare Genetic Variants in Complement Factor I Lead to Low FI Plasma Levels Resulting in Increased Risk of Age-Related Macular Degeneration.
Macular Degeneration
Rare genetic variants in the CFI gene are associated with advanced age-related macular degeneration and commonly result in reduced serum factor I levels.
Macular Degeneration
Support for the involvement of complement factor I in age-related macular degeneration.
Macular Degeneration
[Association of single nucleotide polymorphism in complement factor I gene with age-related macular degeneration].
Malaria
C3b inactivator in normal mice and mice infected with Plasmodium berghei berghei.
Malnutrition
Control of serum C3 levels by beta 1H and C3b inactivator.
Measles
Antibody-independent activation of the alternative complement pathway by measles virus-infected cells.
Meningitis
Complement factor I deficiency associated with recurrent infections, vasculitis and immune complex glomerulonephritis.
Meningitis
Complement factor I deficiency associated with recurrent meningitis coinciding with menstruation.
Meningitis, Aseptic
Complement factor I deficiency with recurrent aseptic meningitis.
Meningococcal Infections
Meningococcal infection and proteolytic control.
Neoplasms
Complement factor I is upregulated in rat hepatocytes by interleukin-6 but not by interferon-gamma, interleukin-1beta, or tumor necrosis factor-alpha.
Neoplasms
New perspectives on role of tumor microenvironment in progression of cutaneous squamous cell carcinoma.
Nephrosis
Correlations between serum factor B and C3b inactivator levels in normal subjects and in patients with infections, nephrosis and hypocomplementaemic glomerulonephritis.
Nephrotic Syndrome
Control of serum C3 levels by beta 1H and C3b inactivator.
Neuroinflammatory Diseases
Classical and Non-classical Presentations of Complement Factor I Deficiency: Two Contrasting Cases Diagnosed via Genetic and Genomic Methods.
Neuroinflammatory Diseases
Mutations of Complement Factor I and Potential Mechanisms of Neuroinflammation in Acute Hemorrhagic Leukoencephalitis.
Osteoarthritis
Alternative pathway complement activation in rheumatoid arthritis.
Osteoarthritis
Expression of proteins in serum, synovial fluid, synovial membrane, and articular cartilage samples obtained from dogs with stifle joint osteoarthritis secondary to cranial cruciate ligament disease and dogs without stifle joint arthritis.
Ovarian Neoplasms
Proteases and their inhibitors as prognostic factors for high-grade serous ovarian cancer.
Parasitemia
The role in vivo of C3 and the C3b receptor in babesial infection in the rat.
Pemphigus
Demonstration of beta 1H globulin in pemphigus.
Peritonitis
Primary Pneumococcal Peritonitis can be the first presentation of a familial complement factor I deficiency.
Pneumococcal Infections
Classical and Non-classical Presentations of Complement Factor I Deficiency: Two Contrasting Cases Diagnosed via Genetic and Genomic Methods.
Pneumococcal Infections
[Complement factor I deficiency revealed by repeated systemic Streptococcus pneumoniae infection]
Pneumonia
C3b inactivator deficiency with immune complex manifestations.
Pre-Eclampsia
Immunological studies on pre-eclampsia in Nigerian women.
Proteinuria
Factor B and C3b inactivator clearance in patients with proteinuria.
Reye Syndrome
Hypocomplementemia in Reye syndrome: relationship to disease stage, circulating immune complexes, and C3b amplification loop protein synthesis.
Rheumatic Diseases
C3b inactivator in the rheumatic diseases. Measurement by radial immunodiffusion and by inhibition of formation of properdin pathway C3 convertase.
Sepsis
Host defense against opportunist microorganisms following trauma. II. Changes in complement and immunoglobulins in patients with abdominal trauma and in septic patients without trauma.
Stomach Neoplasms
Down-regulated expression of complement factor I: a potential suppressive protein for gastric cancer identified by serum proteome analysis.
Thrombotic Microangiopathies
Atypical haemolytic uraemic syndrome: a case of rare genetic mutation.
Thrombotic Microangiopathies
C3 glomerulonephritis and thrombotic microangiopathy of renal allograft after pulmonary infection in a male with concomitant two complement factor I gene variations: a case report.
Thrombotic Microangiopathies
Complement Factor I Mutation May Contribute to Development of Thrombotic Microangiopathy in Lupus Nephritis.
Urticaria
The role of complement in urticaria and angioedema.
Uveitis
CFI-rs7356506 is a genetic protective factor for acute anterior uveitis in Chinese patients.
Uveitis
CFI-rs7356506 polymorphisms associated with Vogt-Koyanagi-Harada syndrome.
Vaccinia
Disabling complement regulatory activities of vaccinia virus complement control protein reduces vaccinia virus pathogenicity.
Vasculitis
Complement factor I deficiency associated with recurrent infections, vasculitis and immune complex glomerulonephritis.
Vasculitis
Cutaneous Vasculitis and Recurrent Infection Caused by Deficiency in Complement Factor I.
Vasculitis
Novel CFI mutation in a patient with leukocytoclastic vasculitis may redefine the clinical spectrum of Complement Factor I deficiency.
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1622
A2G2, deduced molecular weight of N-linked glycan structure
1824
A3G2, deduced molecular weight of N-linked glycan structure
1912
A2G2S1, deduced molecular weight of N-linked glycan structure
2115
A3G2S1, deduced molecular weight of N-linked glycan structure
2202
A2G2S2, deduced molecular weight of N-linked glycan structure
2277
A3G3S1, deduced molecular weight of N-linked glycan structure
2405
A3G2S2, deduced molecular weight of N-linked glycan structure
2567
A3G3S2, deduced molecular weight of N-linked glycan structure
27590
calculated for non-glycosylated light chain, estimated basing upon results of the N-linked glycan analysis
30270
estimated for light chain of a partially deglycosylated factor I bearing a single N-linked Man3GlcNAc2 core structure at each glycosylation site
35290
calculated for non-glycosylated heavy chain, estimated basing upon results of the N-linked glycan analysis
3569
approximate reduction of molecular weight for the heavy chain after partial deglycosylation of native protein with ABS, BTG and GuH exoglycosidases
35900
native light chain, estimated basing upon results from the N-linked glycan analysis
37960
estimated for heavy chain of a partially deglycosylated factor I bearing a single N-linked Man3GlcNAc2 core structure at each glycosylation site
41530
native heavy chain, estimated basing upon results from the N-linked glycan analysis
5632
approximate reduction of molecular weight for the light chain after partial deglycosylation of native protein with ABS, BTG and GuH exoglycosidases
62880
total calculated molecular weight for non-glycosylated factor I, estimated basing upon results of the N-linked glycan analysis
68230
estimated for total protein bearing a single N-linked Man3GlcNAc2 core structure at each glycosylation site
77430
deduced for total protein of the native factor I basing upon results from the N-linked glycan analysis
892
Man3GlcNAc2 core, deduced molecular weight of N-linked glycan structure
90000
unprocessed, under reducing conditions, SDS-PAGE
9201
approximate reduction of molecular weight for total molecule of factor I after partial deglycosylation of native protein with ABS, BTG and GuH exoglycosidases
66000
-
nonglycosylated proenzyme
75000
-
x * 75000, SDS-PAGE
90000
-
Western blotting, unprocessed CFI protein under reducing conditions
additional information
mutant protein reveals a slightly different migration pattern during electrophoresis under reducing conditions, due to proximity of the mutation to a cysteine residue
50000
heavy chain of
50000
heavy chain, indicative of processing
88000
SDS-PAGE
88000
non-reduced CFI proteins, SDS-PAGE
88000
non-reduced form, SDS-PAGE
88000
reduced form, RRKR-linker peptide not cleaved, corresponds to pro-complemement factor I
88000
reduced form, RRKR-linker peptide not cleaved, corresponds to pro-complement factor I
38000
-
SP-domain-form, SDS-PAGE
38000
-
Western blotting, light chain of CFI under reducing conditions
38000
-
light-chain, Western blot, reducing condition
38000
-
1 * 38000 + 1 * 50000, non-catalytic 50000 Da subunit is disulfide-linked to the 38000 Da catalytic subunit
38000
-
1 * 50000 + 1 * 38000, SDS-PAGE, the enzyme is proteolytically processed into the heavy and the light chain that remain covalently linked by a disulfide bond, domains structure overview
50000
-
Western blotting, truncated Q336x mutant under non-reducing conditions and heavy chain of CFI under reducing conditions
50000
-
heavy-chain, Western blot, reducing condition
50000
-
1 * 38000 + 1 * 50000, non-catalytic 50000 Da subunit is disulfide-linked to the 38000 Da catalytic subunit
50000
-
1 * 50000 + 1 * 38000, SDS-PAGE, the enzyme is proteolytically processed into the heavy and the light chain that remain covalently linked by a disulfide bond, domains structure overview
88000
-
sucrose density gradient centrifugation
88000
-
SDS-PAGE, non-reducing conditions
88000
-
coimmunoprecipitation analysis
88000
-
Western blotting, wild-type and the Q232K, C237Y and S250L mutants under non-reducing conditions
88000
-
Western blot, non-reducing condition
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D501N
amino acid exchange in the serine protease domain, resulting in secreted proteins that lack cofactor function of complement components C3b and C4b
D506V
amino acid exchange in the serine protease domain, resulting in secreted proteins that lack cofactor function of complement components C3b and C4b
G243D
cofactor function of complement components C3b and Cb4 not affected
G261D
mutation in the complement factor I heavy chain associated with atypical hemolytic uremic syndrome, recombinant protein generated, activity tested
I322T
amino acid exchange in the serine protease domain, resulting in secreted proteins that lack cofactor function of complement components C3b and C4b
R299W
amino acid exchange in the heavy chain, about 30% activity retained
R388H
cofactor function of complement components C3b and Cb4 not affected
A222G
-
secretion of mutant protein significantly lower compared to wild-type when expressed in human embryonic kidney cells. Mutant cleaves the alpha'-chains of complement factor C4b and C3b as efficiently as wild-type in solution. Compared to wild-type mutant A22G shows impaired cleavage of complement factor C3b on the surface of sheep erythrocytes. Mutant cleaves C3b alpha'chain on the surface of endothelial cells (HUVEV-cells) as efficiently as wild-type
C196S
-
naturally occuring mutation, causes a failure in secretion of the enzyme
C237Y
-
mutation affects secretion, is expressed in smaller amounts as the wild-type. Does not degrade C4b and C3b as efficiently as the wild-type
C25F
-
mutant is as efficiently expressed in human embryonic kidney cells as wild-type, mutant protein is not secreted. Mutant is sensitive to EndoH digestion, indicating that it does not reach the late Golgi compartment and is retained in the endoplasmic reticulum
D104S
-
site-directed mutagenesis, altered kinetics compared to the wild-type
D207N/Q219A
-
Km (tert-butyloxycarbonyl-Asp(benzyl)-Pro-Arg-7-amido-4-methylcoumarin) and Vmax similar to wild-type
D207N/Q219A/M220A/K221Q
-
Km (tert-butyloxycarbonyl-Asp(benzyl)-Pro-Arg-7-amido-4-methylcoumarin) and Vmax similar to wild-type
D26N/K27Q/F29A/Q31A
-
Km (tert-butyloxycarbonyl-Asp(benzyl)-Pro-Arg-7-amido-4-methylcoumarin) similar to wild-type, Vmax significantly increased compared to wild-type. Compared to wild-type mutant shows strongly impaired activity in degradation of fluid-phase complement factor C4b or C3b and in the degradation of surface-bound C3b deposited on sheep erythrocytes
D385N/K387S
-
site-directed mutagenesis, altered kinetics compared to the wild-type
D420N/N422T
-
site-directed mutagenesis, the mutation in the serine protease domain decreases the binding of the enzyme to substrate analogue C3met
D497N
-
site-directed mutagenesis, altered kinetics compared to the wild-type
D501N
-
mutant is as efficiently expressed and secreted in human embryonic kidney cells as wild-type, mutant does not degrade complement factor C4b or C3b. In contrast to wild-type mutant D501N does not cleave C3b alpha'chain on the surface of endothelial cells (HUVEV-cells)
D506V
-
leads to partial IF-deficiency
F29A/Q31A
-
Km (tert-butyloxycarbonyl-Asp(benzyl)-Pro-Arg-7-amido-4-methylcoumarin) similar to wild-type, Vmax significantly increased compared to wild-type. Compared to wild-type mutant shows strongly impaired activity in degradation of fluid-phase complement factor C4b or C3b
F82N/N84T
-
site-directed mutagenesis, the mutation in the FIMAC domain impairs enzyme activity, which is rescued by deglycosylation of the mutant
F94A/K182Q/R184Q
-
Km (tert-butyloxycarbonyl-Asp(benzyl)-Pro-Arg-7-amido-4-methylcoumarin) similar to wild-type, Vmax significantly increased compared to wild-type. Compared to wild-type mutant shows strongly impaired activity in degradation of fluid-phase complement factor C4b or C3b. Mutant shows greatly impaired activity in the degradation of surface-bound C3b deposited on sheep erythrocytes
G170V
-
is expressed at low levels. Does degrade C4b and C3b
G71V
-
naturally occuring mutation, causes a failure in secretion of the enzyme
H165R
-
mutant is as efficiently expressed and secreted in human embryonic kidney cells as wild-type. Mutant cleaves the alpha'-chains of complement factor C4b and C3b more efficiently than wild-type in the presence of C4b-binding protein and factor H as cofactors in solution. Cleavage of complement factor C3b on the surface of sheep erythrocytes is similar to wild-type. In the presence of membrane cofactor protein mutant cleaves C3b alpha'chain on the surface of endothelial cells (HUVEV-cells) more efficiently than wild-type
H400L
-
mutation affects secretion, is expressed at low levels. Does degrade C4b and C3b
I339M
-
mutation affects secretion, is expressed at low levels
K124N
-
site-directed mutagenesis, altered kinetics compared to the wild-type
K124Q/R150Q/F151A/K152Q
-
Km (tert-butyloxycarbonyl-Asp(benzyl)-Pro-Arg-7-amido-4-methylcoumarin) and Vmax similar to wild-type
K182N/R184S
-
site-directed mutagenesis, altered kinetics compared to the wild-type
K182Q/R184Q
-
Km (tert-butyloxycarbonyl-Asp(benzyl)-Pro-Arg-7-amido-4-methylcoumarin) and Vmax similar to wild-type. Mutant shows similar activity to wild-type in degradation of fluid-phase complement factor C4b or C3b and in the degradation of surface-bound C3b deposited on sheep erythrocytes
K326N/A328T
-
site-directed mutagenesis, the mutation in the serine protease domain decreases the binding of the enzyme to substrate analogue C3met
K458N/N460T
-
site-directed mutagenesis, altered kinetics compared to the wild-type
K51A/R62A
-
Km (tert-butyloxycarbonyl-Asp(benzyl)-Pro-Arg-7-amido-4-methylcoumarin) and Vmax similar to wild-type, only mutant in the FI and membrane attack complex domain (FIMAC) which shows some activity in degradation of fluid-phase complement factor C4b or C3b
K51A/R62A/L73A/L76A/F82A
-
Km (tert-butyloxycarbonyl-Asp(benzyl)-Pro-Arg-7-amido-4-methylcoumarin) and Vmax similar to wild-type
K93Q/F94A
-
Km (tert-butyloxycarbonyl-Asp(benzyl)-Pro-Arg-7-amido-4-methylcoumarin) and Vmax similar to wild-type. Compared to wild-type mutant shows decreased but not abolished activity in degradation of fluid-phase complement factor C4b or C3b. Mutant shows impaired activity in the degradation of surface-bound C3b deposited on sheep erythrocytes
L171N
-
site-directed mutagenesis, altered kinetics compared to the wild-type
L289x
-
deletion mutant (c.893delC) leading to a premature stop codon. Mutant protein is not secreted when expressed in human embryonic kidney cells
L73A/L76A/F82A
-
Km (tert-butyloxycarbonyl-Asp(benzyl)-Pro-Arg-7-amido-4-methylcoumarin) and Vmax similar to wild-type
M120V
-
mutant is as efficiently expressed in human embryonic kidney cells as wild-type, secretion is significantly lower compared to wild-type. Mutant cleaves the alpha'-chains of complement factor C4b and C3b more efficiently than wild-type in the presence of C4b-binding protein and factor H as cofactors in solution. Mutant cleaves complement factor C3b more efficiently in the presence of membrane cofactor protein in solution. Compared to wild-type mutant M120V shows enhanced cleavage of complement factor C3b on the surface of sheep erythrocytes. In the presence of membrane cofactor protein mutant cleaves C3b alpha'chain on the surface of endothelial cells (HUVEV-cells) more efficiently than wild-type
M220A/K221Q
-
Km (tert-butyloxycarbonyl-Asp(benzyl)-Pro-Arg-7-amido-4-methylcoumarin) and Vmax similar to wild-type
N133S
-
mutant protein is not secreted when expressed in human embryonic kidney cells, mutant is sensitive to EndoH digestion, indicating that it does not reach the late Golgi compartment and is retained in the endoplasmic reticulum
N404T
-
site-directed mutagenesis, the mutation in the serine protease domain decreases the binding of the enzyme to substrate analogues C3met and C4met
P32A
-
mutant is as efficiently expressed and secreted in human embryonic kidney cells as wild-type. P32A mutant shows impaired function towards degradation of the alpha'-chains of complement factor C4b at the two highest concentrations and of the alpha'-chain of C3b at the highest concentration when factor H is used as cofactor. No significant impairment when complement receptor 1 and membrane cofactor protein 1 are used as cofactors. Compared to wild-type mutant P32A shows impaired cleavage of complement factor C3b on the surface of sheep erythrocytes. Mutant cleaves C3b alpha'chain on the surface of endothelial cells (HUVEV-cells) as efficiently as wild-type
Q210N/V212T
-
site-directed mutagenesis, altered kinetics compared to the wild-type
Q219N/K221S
-
site-directed mutagenesis, altered kinetics compared to the wild-type
Q232K
-
mutation affects secretion, is expressed in smaller amounts as the wild-type. Does degrade C4b and C3b
Q242N/K244S
-
site-directed mutagenesis, the mutation in the LDLr2 domain decreases the binding of the enzyme to substrate analogues C3met and C4met
Q257N/Q259S
-
site-directed mutagenesis, the mutation in the LDLr2 domain decreases the binding of the enzyme to substrate analogues C3met and C4met
Q336x
-
truncated mutant can be expressed, in vitro, at a level similar to that of the wild-type, but is not functional because it lacks the serine protease domain. Is not detected in serum of the patient
R130Q/R169Q
-
Km (tert-butyloxycarbonyl-Asp(benzyl)-Pro-Arg-7-amido-4-methylcoumarin) and Vmax similar to wild-type
R150Q/F151A/K152Q
-
Km (tert-butyloxycarbonyl-Asp(benzyl)-Pro-Arg-7-amido-4-methylcoumarin) and Vmax similar to wild-type
R201S
-
present only in Far East populations with frequencies of about 0.03 in the main island of Japan and lower than 0.01 in Okinawa and Korea
R299W
-
mutant is as efficiently expressed in human embryonic kidney cells as wild-type, secretion is significantly lower compared to wild-type. Mutant cleaves the alpha'-chains of complement factor C4b and C3b as efficiently as wild-type in solution. Cleavage of complement factor C3b on the surface of sheep erythrocytes is similar to wild-type. In the presence of membrane cofactor protein mutant cleaves C3b alpha'chain on the surface of endothelial cells (HUVEV-cells) more efficiently than wild-type
R35N/I37T
-
site-directed mutagenesis, the mutation in the FIMAC domain impairs enzyme activity, which is rescued by deglycosylation of the mutant
R365N
-
site-directed mutagenesis, the mutation in the serine protease domain decreases the binding of the enzyme to substrate analogues C3met and C4met
R406H
-
present almost exclusively in East Asians and at highest frequencies in southern Chinese Han and Thais
R456x
-
point mutant leading to a premature stop codon. Mutant protein is not secreted when expressed in human embryonic kidney cells
R502L
-
must have arisen in a southeastern part of Asia and thereafter has spread to neighboring populations
R61N
-
site-directed mutagenesis, altered kinetics compared to the wild-type
S250L
-
mutation affects secretion, is expressed in smaller amounts as the wild-type. Cleaves C4b and C3b in the same manner as the wild-type
S561N/Y563T
-
site-directed mutagenesis, the mutation in the serine protease domain impairs enzyme activity, which is rescued by deglycosylation of the mutant
T520x
-
insertion mutant (c. 1610insAT) leading to a premature stop codon, mutant protein is not secreted when expressed in human embryonic kidney cells
T54N/V56T
-
site-directed mutagenesis, altered kinetics compared to the wild-type
V212A/L236A
-
Km (tert-butyloxycarbonyl-Asp(benzyl)-Pro-Arg-7-amido-4-methylcoumarin) similar to wild-type, Vmax significantly increased compared to wild-type. Compared to wild-type mutant shows strongly impaired activity in degradation of fluid-phase complement factor C4b or C3b. Mutant shows no activity in the degradation of surface-bound C3b deposited on sheep erythrocytes
V252A/I267A
-
Km (tert-butyloxycarbonyl-Asp(benzyl)-Pro-Arg-7-amido-4-methylcoumarin) similar to wild-type, Vmax significantly increased compared to wild-type. Compared to wild-type mutant shows strongly impaired activity in degradation of fluid-phase complement factor C4b or C3b. Mutant shows no activity in the degradation of surface-bound C3b deposited on sheep erythrocytes
W468x
-
deletion mutant (c.1446-1450 delTTCAC) leading to a premature stop codon, mutant is as efficiently expressed in human embryonic kidney cells as wild-type but protein is not secreted
W528x
-
point mutant leading to a premature stop codon. Mutant protein is not secreted when expressed in human embryonic kidney cells
Y369S
-
patient heterozygous for a novel missense mutation in CFI. This polymorphism is within a functional domain. It is located in the 38 kDa chain of the protein within the serine protease domain that is linked by a disulfide bond to the noncatalytic heavy chain of factor I
M120I
clinical data of aHUS-atients with complement factor I mutation summarized, cofactor function of complement components C3b and Cb4 not affected
M120I
clinical data of aHUS-patients with complemment factor I mutation summarized, cofactor function of complement components C3b and Cb4 not affected
W127X
-
stop-codon, leads to partial IF-deficiency
W127X
-
point mutant leading to a premature stop codon. Mutant protein is not secreted when expressed in human embryonic kidney cells
additional information
deletion mutant delTTCAC (1446-1450) analyzed, no secretion observed
additional information
-
del 922C, leads to partial IF-deficiency
additional information
-
mutations in the FIMAC, CD5, and LDLr1 domains do not decrease the binding of the enzyme to substrate analogues C3met or C4met
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Ekdahl, K.N.; Nilsson, U.R.; Nilsson, B.
Inhibition of factor I by diisopropylfluorophosphate. Evidence of conformational changes in factor I induced by C3b and additional studies on the specificity of factor I
J. Immunol.
144
4269-4274
1990
Homo sapiens
brenda
Medicus, R.G.; Melamed, J.; Arnaout, M.A.
Role of human factor I and C3b receptor in the cleavage of surface-bound C3bi molecules
Eur. J. Immunol.
13
465-470
1983
Homo sapiens
brenda
Hsiung, L.; Barclay, A.N.; Brandon, M.R.; Sim, E.; Porter, R.R.
Purification of human C3b inactivator by monoclonal-antibody affinity chromatography
Biochem. J.
203
293-298
1982
Homo sapiens
brenda
Crossley, L.G.
C3b inactivator and beta1H
Methods Enzymol.
80
112-124
1981
Homo sapiens
brenda
Nagasawa, S.; Ichihara, C.; Stroud, R.M.
Cleavage of C4b by C3b inactivator: production of a nicked form of C4b, C4b', as an intermediate cleavage product of C4b by C3b inactivator
J. Immunol.
125
578-582
1980
Homo sapiens
brenda
Fujita, T.; Nussenzweig, V.
The role of C4-binding protein and beta1H in proteolysis of C4b and C3b
J. Exp. Med.
150
267-276
1979
Homo sapiens
brenda
Gaither, T.A.; Hammer, C.H.; Frank, M.M.
Studies of the molecular mechanisms of C3b inactivation and a simplified assay of beta1H and the C3b inactivator (C3bINA)
J. Immunol.
123
1195-1204
1979
Homo sapiens
brenda
DiScipio, R.G.
The fractionation of human plasma proteins
Protein Expr. Purif.
5
178-186
1994
Homo sapiens
brenda
Van den Berg, C.W.; Perez de la Lastra, J.M.; Llanes, D.; Morgan, B.P.
Purification and characterization of the pig analogue of human membrane cofactor protein (CD46/MCP)
J. Immunol.
158
1703-1709
1997
Homo sapiens, Sus scrofa
brenda
Ekdahl, K.N.; Nilsson, B.
Phosphorylation of complement component C3 and C3 fragments by a human platelet protein kinase
J. Immunol.
154
6402-6510
1995
Homo sapiens
-
brenda
Cunnion, K.M.; Hair, P.S.; Buescher, E.S.
Cleavage of complement C3b to iC3b on the surface of Staphylococcus aureus is mediated by serum complement factor I
Infect. Immun.
72
2858-2863
2004
Homo sapiens
brenda
Tsiftsoglou, S.A.; Sim, R.B.
Human complement factor I does not require cofactors for cleavage of synthetic substrates
J. Immunol.
173
367-375
2004
Homo sapiens
brenda
Tsiftsoglou, S.A.; Willis, A.C.; Li, P.; Chen, X.; Mitchell, D.A.; Rao, Z.; Sim, R.B.
The catalytically active serine protease domain of human complement factor I
Biochemistry
44
6239-6249
2005
Homo sapiens
brenda
Kavanagh, D.; Kemp, E.J.; Mayland, E.; Winney, R.J.; Duffield, J.S.; Warwick, G.; Richards, A.; Ward, R.; Goodship, J.A.; Goodship, T.H.
Mutations in complement factor I predispose to development of atypical hemolytic uremic syndrome
J. Am. Soc. Nephrol.
16
2150-2155
2005
Homo sapiens
brenda
Cunnion, K.M.; Buescher, E.S.; Hair, P.S.
Serum complement factor I decreases Staphylococcus aureus phagocytosis
J. Lab. Clin. Med.
146
279-286
2005
Homo sapiens
brenda
Tsiftsoglou, S.A.; Arnold, J.N.; Roversi, P.; Crispin, M.D.; Radcliffe, C.; Lea, S.M.; Dwek, R.A.; Rudd, P.M.; Sim, R.B.
Human complement factor I glycosylation: structural and functional characterisation of the N-linked oligosaccharides
Biochim. Biophys. Acta
1764
1757-1766
2006
Homo sapiens (P05156), Homo sapiens
brenda
Nilsson, S.C.; Karpman, D.; Vaziri-Sani, F.; Kristoffersson, A.C.; Salomon, R.; Provot, F.; Fremeaux-Bacchi, V.; Trouw, L.A.; Blom, A.M.
A mutation in factor I that is associated with atypical hemolytic uremic syndrome does not affect the function of factor I in complement regulation
Mol. Immunol.
44
1835-1844
2007
Homo sapiens (P05156)
brenda
Timar, K.K.; Junnikkala, S.; Dallos, A.; Jarva, H.; Bhuiyan, Z.A.; Meri, S.; Bos, J.D.; Asghar, S.S.
Human keratinocytes produce the complement inhibitor factor I: Synthesis is regulated by interferon-gamma
Mol. Immunol.
44
2943-2949
2007
Homo sapiens (P05156), Homo sapiens
brenda
Okroj, M.; Hsu, Y.F.; Ajona, D.; Pio, R.; Blom, A.M.
Non-small cell lung cancer cells produce a functional set of complement factor I and its soluble cofactors
Mol. Immunol.
45
169-179
2008
Homo sapiens (P05156)
brenda
Kavanagh, D.; Richards, A.; Noris, M.; Hauhart, R.; Liszewski, M.K.; Karpman, D.; Goodship, J.A.; Fremeaux-Bacchi, V.; Remuzzi, G.; Goodship, T.H.; Atkinson, J.P.
Characterization of mutations in complement factor I (CFI) associated with hemolytic uremic syndrome
Mol. Immunol.
45
95-105
2008
Homo sapiens (P05156)
brenda
Chan, M.R.; Thomas, C.P.; Torrealba, J.R.; Djamali, A.; Fernandez, L.A.; Nishimura, C.J.; Smith, R.J.; Samaniego, M.D.
Recurrent atypical hemolytic uremic syndrome associated with factor I mutation in a living related renal transplant recipient
Am. J. Kidney Dis.
53
321-326
2009
Homo sapiens
brenda
Fagerness, J.A.; Maller, J.B.; Neale, B.M.; Reynolds, R.C.; Daly, M.J.; Seddon, J.M.
Variation near complement factor I is associated with risk of advanced AMD
Eur. J. Hum. Genet.
17
100-104
2009
Homo sapiens
brenda
Nilsson, S.C.; Trouw, L.A.; Renault, N.; Miteva, M.A.; Genel, F.; Zelazko, M.; Marquart, H.; Muller, K.; Sjoeholm, A.G.; Truedsson, L.; Villoutreix, B.O.; Blom, A.M.
Genetic, molecular and functional analyses of complement factor I deficiency
Eur. J. Immunol.
39
310-323
2009
Homo sapiens
brenda
Yuasa, I.; Nakagawa, M.; Umetsu, K.; Harihara, S.; Matsusue, A.; Nishimukai, H.; Fukumori, Y.; Saitou, N.; Park, K.S.; Jin, F.; Lucotte, G.; Chattopadhyay, P.K.; Henke, L.; Henke, J.
Molecular basis of complement factor I (CFI) polymorphism: one of two polymorphic suballeles responsible for CFI A is Japanese-specific
J. Hum. Genet.
53
1016-1021
2008
Homo sapiens
brenda
Wang, J.; Ohno-Matsui, K.; Yoshida, T.; Kojima, A.; Shimada, N.; Nakahama, K.; Safranova, O.; Iwata, N.; Saido, T.C.; Mochizuki, M.; Morita, I.
Altered function of factor I caused by amyloid beta: implication for pathogenesis of age-related macular degeneration from Drusen
J. Immunol.
181
712-720
2008
Homo sapiens, Mus musculus
brenda
Hair, P.S.; Ward, M.D.; Semmes, O.J.; Foster, T.J.; Cunnion, K.M.
Staphylococcus aureus clumping factor A binds to complement regulator factor I and increases factor I cleavage of C3b
J. Infect. Dis.
198
125-133
2008
Homo sapiens
brenda
Ponce-Castro, I.M.; Gonzalez-Rubio, C.; Delgado-Cervino, E.M.; Abarrategui-Garrido, C.; Fontan, G.; Sanchez-Corral, P.; Lopez-Trascasa, M.
Molecular characterization of Complement Factor I deficiency in two Spanish families
Mol. Immunol.
45
2764-2771
2008
Homo sapiens
brenda
Nakahata, K.; Matsunami, K.; Kobayashi, C.; Omori, T.; Xu, H.; Firdawes, S.; Fukuzawa, M.; Miyagawa, S.
Analysis of the serine protease function of porcine factor I produced by liver cells for xenotransplantation
Transpl. Immunol.
19
30-36
2008
Homo sapiens (P05156), Homo sapiens, Sus scrofa
brenda
Nilsson, S.C.; Kalchishkova, N.; Trouw, L.A.; Fremeaux-Bacchi, V.; Villoutreix, B.O.; Blom, A.M.
Mutations in complement factor I as found in atypical hemolytic uremic syndrome lead to either altered secretion or altered function of factor I
Eur. J. Immunol.
40
172-185
2010
Homo sapiens
brenda
Hair, P.S.; Echague, C.G.; Sholl, A.M.; Watkins, J.A.; Geoghegan, J.A.; Foster, T.J.; Cunnion, K.M.
Clumping factor A interaction with complement factor I increases C3b cleavage on the bacterial surface of Staphylococcus aureus and decreases complement-mediated phagocytosis
Infect. Immun.
78
1717-1727
2010
Homo sapiens
brenda
Ennis, S.; Gibson, J.; Cree, A.; Collins, A.; Lotery, A.
Support for the involvement of complement factor I in age-related macular degeneration
Eur. J. Hum. Genet.
18
15-16
2010
Homo sapiens
brenda
Roversi, P.; Johnson, S.; Caesar, J.J.; McLean, F.; Leath, K.J.; Tsiftsoglou, S.A.; Morgan, B.P.; Harris, C.L.; Sim, R.B.; Lea, S.M.
Structural basis for complement factor I control and its disease-associated sequence polymorphisms
Proc. Natl. Acad. Sci. USA
108
12839-12844
2011
Homo sapiens
brenda
Sanchez-Gallego, J.I.; Groeneveld, T.W.; Krentz, S.; Nilsson, S.C.; Villoutreix, B.O.; Blom, A.M.
Analysis of binding sites on complement factor I using artificial N-linked glycosylation
J. Biol. Chem.
287
13572-13583
2012
Homo sapiens
brenda
Broderick, L.; Gandhi, C.; Mueller, J.L.; Putnam, C.D.; Shayan, K.; Giclas, P.C.; Peterson, K.S.; Aceves, S.S.; Sheets, R.M.; Peterson, B.M.; Newbury, R.O.; Hoffman, H.M.; Bastian, J.F.
Mutations of complement factor I and potential mechanisms of neuroinflammation in acute hemorrhagic leukoencephalitis
J. Clin. Immunol.
33
162-171
2013
Homo sapiens
brenda
Malm, S.; Jusko, M.; Eick, S.; Potempa, J.; Riesbeck, K.; Blom, A.M.
Acquisition of complement inhibitor serine protease factor I and its cofactors C4b-binding protein and factor H by Prevotella intermedia
PLoS ONE
7
e34852
2012
Homo sapiens
brenda
Okroj, M.; Holmquist, E.; Nilsson, E.; Anagnostaki, L.; Jirstroem, K.; Blom, A.M.
Local expression of complement factor I in breast cancer cells correlates with poor survival and recurrence
Cancer Immunol. Immunother.
64
467-478
2015
Homo sapiens
brenda
Kumar, J.; Yadav, V.; Phulera, S.; Kamble, A.; Gautam, A.; Panwar, H.; Sahu, A.
Species specificity of vaccinia virus complement control protein for the bovine classical pathway is governed primarily by direct interaction of its acidic residues with factor I
J. Virol.
91
e00668-17
2017
Bos taurus, Homo sapiens
brenda