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Product Description
C1 Inhibitor 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. C1inhibitor can also inhibit various other proteases, including Kallikrein, Factor XIa, and Factor XIIa. Deficiencies in C1inhibitor 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 C1inhibitor can be used to treat HAE and certain other conditions. Recombinant Human C1 Inhibitor is a highly glycosylated glycoprotein containing 445 amino acid residues (49.4kDa), corresponding to amino acids 56 500 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 approximately 80-90 kDa by SDS PAGE analysis under reducing conditions.
Biovision | 7120 | C1 Inhibitor Human Recombinant DataSheet
Biomolecule/Target: C1 inhibitor
Synonyms: Plasma protease C1, inhibitor, C1inh, C1 Esterase Protein, C1-inhibiting factor, Serpin G1
Alternates names: Beta-defensin 3, Defensin, beta 103, Defensin-like protein, DEFB103A, BD3, DEFB103, DEFB3, DEFB103B
Taglines: One of the primary regulators of the immune complement system
NCBI Gene ID #: 140596
NCBI Gene Symbol: DEFB4
Gene Source: Human
Accession #: Q8WTQ1
Recombinant: Yes
Source: CHO cells
Purity by SDS-PAGEs: 98%
Assay: SDS-PAGE
Purity: N/A
Assay #2: HPLC
Endotoxin Level: < 0.1 ng/g of protein (<1EU/g).
Activity (Specifications/test method): Measured by its ability to inhibit recombinant human complement component C1a cleavage of a colorimetric peptide substrate, N Carbobenzyloxy-LysThioBenzyl ester (Z-K-SBzl). The expected IC50 is 2.6 nM
Biological activity: Determined by its ability to chemoattract human monocytes using a concentration range of 0.1-50 ng/ml.
Results: N/A
Binding Capacity: N/A
Unit Definition: N/A
Molecular Weight: ~6.0 kDa
Concentration: N/A
Appearance: Lyophilized powder
Physical form description: Sterile filtered through a 0.2 micron filter. Lyophilized with 10 mM sodium phosphate, pH 7.5.
Reconstitution Instructions: Centrifuge the vial prior to opening. Reconstitute in sterile dd HO to a concentration 100 µg/ml. This solution can then be diluted into other aqueous buffers and stored at 4 °C for 2-7 days and at -20 °C for future use.
Amino acid sequence: N/A
