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Complement Factor I (CFI)

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CF-I; C3b-INA; FI; IF; KAF; Factor I; Konglutinogen-Activating Factor; C3b-Inactivator; C3B/C4B inactivator

Complement Factor I (CFI)
CCC B978
HGNC 5394
MGI 105937
PubMed 7922290
RGD 620429
UniProt P05156
Wiki CFI

Factor I is 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.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPB978Hu01 Recombinant Complement Factor I (CFI) Positive Control; Immunogen; SDS-PAGE; WB.
RPB978Hu02 Recombinant Complement Factor I (CFI) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAB978Hu01 Polyclonal Antibody to Complement Factor I (CFI) WB; IHC; ICC; IP.
MAB978Hu22 Monoclonal Antibody to Complement Factor I (CFI) WB; IHC; ICC; IP.
PAB978Hu02 Polyclonal Antibody to Complement Factor I (CFI) WB; IHC; ICC; IP.
Assay Kits SEB978Hu ELISA Kit for Complement Factor I (CFI) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPB978Mu01 Recombinant Complement Factor I (CFI) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAB978Mu01 Polyclonal Antibody to Complement Factor I (CFI) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Complement Factor I (CFI) CLIA Kit Customized Service Offer
n/a ELISA Kit for Complement Factor I (CFI) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Complement Factor I (CFI) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Complement Factor I (CFI) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Complement Factor I (CFI) Polyclonal Antibody Customized Service Offer
Assay Kits SEB978Ra ELISA Kit for Complement Factor I (CFI) Enzyme-linked immunosorbent assay for Antigen Detection.
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  2. "Human complement factor I: analysis of cDNA-derived primary structure and assignment of its gene to chromosome 4."J. Biol. Chem. 262:10065-10071(1987) [PubMed] [Europe PMC] [Abstract]
  3. "Generation and annotation of the DNA sequences of human chromosomes 2 and 4." Nature 434:724-731(2005) [PubMed] [Europe PMC] [Abstract]
  4. "Cloning and characterization of the promoter for the human complement factor I (C3b/C4b inactivator) gene."Gene 208:17-24(1998) [PubMed] [Europe PMC] [Abstract]
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  11. "Structural basis for complement factor I control and its disease-associated sequence polymorphisms."Proc. Natl. Acad. Sci. U.S.A. 108:12839-12844(2011) [PubMed] [Europe PMC] [Abstract]
  12. "The molecular basis of hereditary complement factor I deficiency."J. Clin. Invest. 97:925-933(1996) [PubMed] [Europe PMC] [Abstract]
  13. "Molecular characterization of homozygous hereditary factor I deficiency."Clin. Exp. Immunol. 131:280-286(2003) [PubMed] [Europe PMC] [Abstract]
  14. "Complement factor I: a susceptibility gene for atypical haemolytic uraemic syndrome."J. Med. Genet. 41:E84-E84(2004) [PubMed] [Europe PMC] [Abstract]
  15. "Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome."Blood 108:1267-1279(2006) [PubMed] [Europe PMC] [Abstract]
  16. "Primary glomerulonephritis with isolated C3 deposits: a new entity which shares common genetic risk factors with haemolytic uraemic syndrome."J. Med. Genet. 44:193-199(2007) [PubMed] [Europe PMC] [Abstract]
  17. "A missense mutation in factor I (IF) predisposes to atypical haemolytic uraemic syndrome."Pediatr. Nephrol. 22:371-375(2007) [PubMed] [Europe PMC] [Abstract]
  18. "Mutations in alternative pathway complement proteins in American patients with atypical hemolytic uremic syndrome."Hum. Mutat. 31:E1445-E1460(2010) [PubMed] [Europe PMC] [Abstract]
  19. "A functional variant in the CFI gene confers a high risk of age-related macular degeneration." Nat. Genet. 45:813-817(2013) [PubMed] [Europe PMC] [Abstract]
  20. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]