Description
This gene is a member of the Regulator of Complement Activation (RCA) gene cluster and encodes a protein with twenty short consensus repeat (SCR) domains. This protein is secreted into the bloodstream and has an essential role in the regulation of complement activation, restricting this innate defense mechanism to microbial infections. Mutations in this gene have been associated with hemolytic-uremic syndrome (HUS) and chronic hypocomplementemic nephropathy. Alternate transcriptional splice variants, encoding different isoforms, have been characterized.
Bio-activity
Measured by the ability of the immobilized protein to induce the adhesion of human neutrophils. DiScipio, R.G. et al. (1998) J. Immunol. 160:4057. The ED50 for this effect is 2.5-10 μg/mL in the presence of 50 ng/mL of rhTNF-alpha.
Storage
Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
12 months from date of receipt, -20 to -70 centigrade as supplied.
1 month, 2 to 8 centigrade under sterile conditions after reconstitution.
3 months, -20 to -70 centigrade under sterile conditions after reconstitution.
