It is thought that the complement system might play a role in many diseases with an immune component, such as Alzheimers disease, asthma, lupus erythematosus, various forms of arthritis, autoimmune heart disease and multiple sclerosis. Deficiencies of the terminal pathway predispose to both autoimmune disease and infections (particularly meningitis) Human Complement C4c is used as an immunogen and is an inactive form. C4c is produced in human plasma having a molecular mass of 210kD. It interacts with complement C1 and complement C2 to form complement C3 convertase of the classic activation pathway. Systemic lupus erythematosus is often associated with congenital complement C4 deficiency. Reduced levels of components of the classic pathway complement(s)(C1, C2, C4, C3) are common after such activation, Systemic lupus erythematosus , acute serum sickness and conditions associated with circulating immune complexes. Complement C4 (200kD) is composed of three polypeptide chains - alpha (93kD), beta (78kD), and gamma (33kD). During complement activation, C4 is cleaved by the serine protease C1s to C4a (9kD) and C4b (190kD). C4b is further cleaved by factor I to C4d (45kD) and C4c (140kD). The current protocol yields C4c, however intact C4 and/or C4b and C4d may be co-purified. Protein: As Reported Identity: Confirmed by Western Blot Storage and Stability: Lyophilized and reconstituted products are stable for 6 months after receipt at -20C. Reconstitute with sterile ddH2O. Aliquot to avoid repeated freezing and thawing. Store at -20C. 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.
Reinheit:
95% (SDS-PAGE)
Formulierung:
Supplied as a lyophilized powder in PBS, pH 7.4.
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