C1 Inhibitor, Recombinant, Human (Plasma Protease C1 Inhibitor, C1 Inh, C1Inh, C1 Esterase Inhibitor, C1-inhibiting Factor, Serpin G1, C1IN, C1NH)

C1Inhibitor is a member of the serpin family of structurally related proteins, and is the primary regulator of the immune complement system. C1 Inhibitor is a protease inhibitor that functions to inhibit the complement system in order to prevent over-activation or spontaneous activation. Inhibition is achieved by binding to and irreversibly inhibiting the C1r and C1s proteases of the C1 complex, which has the effect of shutting down all subsequent downstream events in the complement activation cascade. C1 inhibitor can also inhibit various other proteases, including Kallikrein, Factor XIa, and Factor XIIa. Deficiencies in C1 inhibitor are the primary cause of hereditary angioedema (HAE, hereditary angioneurotic edema), a disease characterized by edema in the respiratory and gastrointestinal tracts. In certain clinical situations, the direct administration of C1 inhibitor can be used to treat HAE and certain other conditions. Recombinant Human C1 Inhibitor is a highly glycosylated glycoprotein containing 445aa residues (49.4kD), corresponding to aa56-500 of the C1 inhibitor precursor, and is fully functional in its ability to inhibit the C1 complex. Glycosylated C1 Inhibitor migrates at an apparent molecular weight of ~80-90kD by SDS PAGE analysis under reducing conditions.\n\nBiological Activity:\nMeasured by its ability to inhibit recombinant human complement component C1a cleavage of a colorimetric peptide substrate, N Carbobenzyloxy-Lys-ThioBenzyl ester (Z-K-SBzl). The expected IC50 is ~2.6nM.\n\nAA Sequence:\nVEPILEVSSL PTTNSTTNSA TKITANTTDE PTTQPTTEPT TQPTIQPTQP TTQLPTDSPT QPTTGSFCPG PVTLCSDLES HSTEAVLGDA LVDFSLKLYH AFSAMKKVET NMAFSPFSIA SLLTQVLLGA GENTKTNLES ILSYPKDFTC VHQALKGFTT KGVTSVSQIF HSPDLAIRDT FVNASRTLYS SSPRVLSNNS DANLELINTW VAKNTNNKIS RLLDSLPSDT RLVLLNAIYL SAKWKTTFDP KKTRMEPFHF KNSVIKVPMM NSKKYPVAHF IDQTLKAKVG QLQLSHNLSL VILVPQNLKH RLEDMEQALS PSVFKAIMEK LEMSKFQPTL LTLPRIKVTT SQDMLSIMEK LEFFDFSYDL NLCGLTEDPD LQVSAMQHQT VLELTETGVE AAAASAISVA RTLLVFEVQQ PFLFVLWDQQ HKFPVFMGRV YDPRA\n\nQuality Control:\nVerified by N-terminal and Mass Spectrometry analyses (when applicable).\n\nEndotoxin:\n<0.1ng/ug of protein (<1EU/ug).\n\nProtein Content:\nVerified by UV Spectroscopy and/or SDS-PAGE gel.\n\nStorage and Stability:\nStore lyophilized products at -20 degrees C. For reconstituted solutions of most products, we recommend short-term storage at 4 degrees C. For longer term storage the protein solution should be stored with a carrier protein (eg. 0.1% BSA) in working aliquots and stored frozen at -20 degrees C. Additional freeze/thaw cycles may cause some denaturation of the protein.

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