catalog number :
MBS851348
products full name :
CFI Antibody
products short name :
[CFI]
products name syn :
[C3b-INA; C3B/C4B inactivator; CFAI; complement factor I; factor I; FI; I factor (complement); IF; KAF; light chain of factor I]
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]
specificity :
CFI antibody detects endogenous levels of total CFI protein.
purity :
Affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
form :
In phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
storage stability :
Store at-20 degree C for 1 year.
tested application :
Western Blot (WB), Immunohistochemistry (IHC), ELISA (EIA)
app notes :
WB: 1:500~1:1000. IHC: 1:50~1:100. ELISA: 1:10000
image1 heading :
Western Blot (WB)
other info1 :
Immunogen: The antiserum was produced against synthesized peptide derived from internal of human CFI.
products categories :
Autophagy antibody; Cancer; Cardiovascular; Cell Biology; Epigenetics & Nuclear Signaling; Developmental Biologys; Immunology; Drug Discovery Products; Metabolism; Neuroscience; Signal Transduction; Stem Cells
products references :
Wilson R.K., Nature 434:724-731(2005). Goldberger G., Gene 208:17-24(1998). Wang L., Zou H., J. Proteome Res. 8:651-661(2009).
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.