84-4411-30　CFI (Complement Factor I, CF-I, C3b-INA, FI, IF, KAF, Factor I, Konglutinogen-Activating Factor, C3b-Inactivator, C3B/C4B inactivator) 200ul　362537

特徴

• Factor I is 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.
• Applications:Suitable for use in ELISA, Western Blot, Immunohistochemistry and Immunocytochemistry.
• Other applications not tested.
• Recommended Dilutions:Western Blot: 0.5-2ug/ml Immunohistochemistry (Paraffin): 5-20ug/mlImmunocytochemistry: 5-20ug/ml Optimal dilutions to be determined by the researcher.
• Positive Control Included:362537A: CFI, Positive ControlSuppled as a recombinant protein in loading buffer (100mM Tris, pH 6.8, 1% SDS, 150mM sodium chloride, 50mM DTT, 0.02% BPB, 0.02% sodium azide, 50% glycerol)
• DAB: 10ul/wellECL: 5ul/wellStore at -20°C.
• Stable for 6 months after receipt.
• Storage and Stability:May be stored at 4°C for short-term only.
• Aliquot to avoid repeated freezing and thawing.
• Store at -20°C.
• Aliquots are stable for 12 months after receipt.
• For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.