Western blot analysis of extracts of Human serum, using CFI antibody (A5623) at 1:500 dilution.|Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution.|Lysates/proteins: 25ug per lane.|Blocking buffer: 3% nonfat dry milk in TBST.|Detection: ECL Basic Kit (RM00020).|Exposure time: 5s.
CFI Rabbit pAb
Catalog No:AB-A5623
Manufacturer:ABclonal
Synonyms:CFI;AHUS3;ARMD13;C3BINA;C3b-INA;FI;IF;KAF
Applications:WB,IF
Reactivity:Human,Mouse
Host:Rabbit
Conjugate:No
€65,00 – €370,00
Product Name: | CFI Rabbit pAb |
Category: | Polyclonal Antibodies |
Description: | 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. |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 19-300 of human CFI (NP_000195.2). |
Gene ID: | 3426 |
Swiss prot: | P05156 |
Molecular weight: | 65kDa |
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