Complement Factor I / Unconjugated / 17F1

Product Details
Supplier LifeSpan
Catalog #: LS-C339810 (View supplier product page)
Size 100 μl
Price
Antigen Complement Factor I
Clone 17F1
Host Mouse
Isotype IgG1
Conjugate Unconjugated
Target Species Human
Applications WB
Description CFI / Complement Factor I Antibody (aa340-583, clone 17F1)
About Complement Factor I and Purified
Complement Factor I 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]
Citations
Additional
Sources
Reviews & Ratings
Purified Excitation and Emission Spectra