CFI Polyclonal Antibody
| Code | Size | Price |
|---|
| E-AB-62790-60uL | 60uL | £210.00 |
Quantity:
| E-AB-62790-120uL | 120uL | £291.00 |
Quantity:
| E-AB-62790-200uL | 200uL | £431.00 |
Quantity:
Prices exclude any Taxes / VAT
Overview
Host Type: Rabbit
Antibody Isotype: IgG
Antibody Clonality: Polyclonal
Regulatory Status: RUO
Target Species:
- Human
- Mouse
Application: Western Blot (WB)
Shipping:
Ice packs
Storage:
Store at -20°C. Avoid freeze / thaw cycles.
Further Information
Abbreviation:
CFI
Background:
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.
Buffer:
PBS with 0.02% sodium azide,50% glycerol,pH7.3.
Calculated MW:
65kDa
Concentration:
1 mg/mL
Conjugation:
Unconjugated
Dilution:
WB 1:500-1:2000
Immunogen:
Recombinant fusion protein of human CFI
ObservedMW:
60kDa
Purification method:
Affinity purification
Target Synonym:
CFI;AHUS3;ARMD13;C3BINA;C3b-INA;FI;IF;KAF
UNIProt ID:
P05156