84-1176-68　C1 Esterase Inhibitor (C1IN, C1 Inh, C1Inh, C1-INH, C1NH, C1-inhibiting Factor, HAE1, HAE2, Plasma Protease C1 Inhibitor, Serpin G1, SERPING1) 1mg　C0003-01E

特徴

• C1-INH is a member of the serpin family of proteases, as are alpha-antitrypsin, antithrombin III, and angiotensinogen.
• These proteins stoichiometrically inactivate their target proteases by forming stable, one-to-one complexes with the protein to be inhibited.
• Although synthesized primarily by hepatocytes, C1-INH is also synthesized by monocytes.
• The regulation of the protein production is not completely understood, but, since patients respond clinically to androgen therapy serum levels of C1-INH increase, it is believed that androgens may stimulate C1-INH synthesis.
• All C1-INH deficient patients are heterozygous; half the normal level of C1-INH is not believed sufficient to prevent attacks.
• Although named for its action on the first component of complement (C1 esterase), C1-INH also inhibits components of the fibrinolytic, clotting, and kinin pathways.
• Specifically, C1-INH inactivates plasmin-activated Hageman factor (factor XII), activated factor XI, PTA, and kallikrein.
• Within the complement system, C1-INH blocks the activation of C1 and the rest of the classic complement pathway by binding to C1r and C1s.
• Without C1-INH, unchecked activation of C1, C2, and C4 occur before other inhibitors (C4-binding protein and factor I) can halt the cascade.
• The actual factor or factors responsible for the edema formation remain somewhat controversial.
• Researchers have demonstrated activation of the kinin system and increased bradykinin concentration associated with clinical flares.
• Applications:Suitable for use in ELISA, Western Blot, Immunocytochemistry, Immunohistochemistry, Immunofluorescence and FACS.
• Other applications not tested.
• Recommended Dilutions:Optimal dilutions to be determined by the researcher.
• 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.