A positive regulator of the alternate pathway of complement. It binds to and stabilizes the C3- and C5-convertase enzyme complexes.
Involvement in disease:Defects in CFP are the cause of properdin deficiency (PFD). PFD results in higher susceptibility to bacterial infections; especially to meningococcal infections. Three phenotypes have been reported: complete deficiency (type I), incomplete deficiency (type II), and dysfunction of properdin (type III).
Images
Western blot analysis of Properdin (GB112049) at dilution of 1: 1000
Lane 1: Mouse plasma tissue lysate
Lane 2: Rat plasma tissue lysate
Storage
Storage
Store at -20 ℃ for one year. Avoid repeated freeze/thaw cycles.
Storage Buffer
PBS with 0.02%sodium azide,100 μg/ml BSA and 50% glycerol.
NOTE:
1.This product is intended for research only.
2.This product is recommended to dilute with the Primary Antibody Dilution Buffer .