Factor I antibody [3R/8]

Catalogue Number: GTX41627-GTX

Manufacturer:GeneTex
Preservative:0.1% Sodium azide
Molecular Weight:66
Physical state:Liquid
Type:Monoclonal Primary Antibody - Unconjugated
Alias:complement factor I , AHUS3 , ARMD13 , C3BINA , C3b-INA , FI , IF , KAF
Shipping Condition:Blue Ice
Unit(s): 100 ug
Host name: Mouse
Clone: 3R/8
Isotype: IgG1
Immunogen: Native human factor I from serum.
Application: ELISA, WB, IHC-Fr, NT, Inh

Description

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. [provided by RefSeq, Dec 2015]

Additional Text

Uniprot ID

P05156

Gene ID

3426

Gene Name

CFI

Molecular Weight

66

Purification

Protein A purified

Antibody Clonality

Monoclonal

Note

For In vitro laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption

Concentration

1 mg/ml

Application Notes

ELISA: 1/500-1/4,000. Neutralizing/Inhibition: . *Optimal dilutions/concentrations should be determined by the researcher.Not tested in other applications.

Storage Note

Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4C. For long-term storage, aliquot and store at -20C or below. Avoid multiple freeze-thaw cycles.