BD-PE0129

CFI (17R18) Mouse Monoclonal antibody

常规抗体

CFI

WB

Human

PBS (pH 7.3) containing 1% BSA, 50% glycerol and 0.02% New type preservative N.

Human recombinant protein fragment corresponding to amino acids 340-583 of human CFI(NP_000195) produced in E.coli.

WB 1:2000

63.4kDa

-20°C/1 year

Mouse

IgG1

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 uraemic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immmune deposits is another condition associated with mutation of this gene. [provided by RefSeq, Jul 2008]

现货

Purified from mouse ascites fluids or tissue culture supernatant by affinity chromatography (protein A/G)