catalog number :
MBS7104812
products full name :
CFI Antibody, Biotin conjugated
products short name :
[CFI]
products name syn :
[Complement factor I; C3B/C4B inactivator; Complement factor I heavy chain; Complement factor I light chain; CFI; IF]
other names :
[complement factor I isoform 2 preproprotein; Complement factor I; complement factor I; complement factor I; C3B/C4B inactivator]
products gene name :
[CFI]
other gene names :
[CFI; CFI; FI; IF; KAF; AHUS3; ARMD13; C3BINA; C3b-INA; IF]
purity :
>95%, Protein G purified
storage stability :
Upon receipt, store at -20 degree C or -80 degree C. Avoid repeated freeze.
tested application :
ELISA (EIA)
other info1 :
Immunogen: Recombinant Human Complement factor I protein (239-316AA)
other info2 :
Conjugation: Biotin. Buffer: Preservative: 0.03% Proclin 300. Constituents: 50% Glycerol, 0.01M PBS, pH 7.4. Species: Homo sapiens (Human). Research Area: Immunology
products categories :
Immunology
products description :
Responsible for cleaving the alpha-chains of C4b and C3b in the presence of the cofactors C4-binding protein and factor H respectively.
ncbi acc num :
NP_000195.2
ncbi gb acc num :
NM_000204.4
ncbi pathways :
Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); Complement Cascade Pathway (106405); Immune System Pathway (106386); Innate Immune System Pathway (106387); Regulation Of Complement Cascade Pathway (576254); Staphylococcus Aureus Infection Pathway (172846); Staphylococcus Aureus Infection Pathway (171867)
ncbi summary :
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 uremic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immune deposits and age-related macular degeneration are other conditions associated with mutations of this gene. [provided by RefSeq, Dec 2015]
uniprot summary :
Responsible for cleaving the alpha-chains of C4b and C3b in the presence of the cofactors C4-binding protein and factor H respectively.