84-4375-59　Factor I (Complement Factor I, AHUS3, ARMD13, C3b INA, C3BINA, C3b Inactivator, C3B/C4B Inactivator, Cfi, Complement Component I, Complement Factor, KAF) 100ug　353257

特徴

• Complement factor I, also known as C3b/C4b inactivator, is a protein that in humans is encoded by the CFI gene.
• 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.
• Applications:Suitable for use in Western Blot.
• Other applications not tested.
• Recommended Dilution:Western Blot: 0.1-0.5ug/mlOptimal dilutions to be determined by the researcher.
• Storage and Stability:Lyophilized powder may be stored at -20°C.
• Reconstitute with sterile ddH2O.
• Aliquot to avoid repeated freezing and thawing.
• Store at -20°C.
• For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
• Further dilutions can be made in assay buffer.