The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/8000. (based on use as detection antibody for antigen coated at 0.3 µg/ml).
Use at an assay dependent dilution. Recognises both reduced and unreduced forms of the protein.
Use at an assay dependent dilution. Suitable for affinity purification of human
properdin after coupling to an activated CNBr-sepharose column.
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) [MIM:312060]. 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).