Alternative complement pathway activity in sera from patients with sickle cell disease. Clin Exp Immunol 1976 Jan;23(1):56-60
Date
01/01/1976Pubmed ID
816581Pubmed Central ID
PMC1538371Scopus ID
2-s2.0-0016892577 (requires institutional sign-in at Scopus site) 48 CitationsAbstract
The low molecular weight cobra venom factor (CoVF) was used to activate the terminal sequence of the alternative complement pathway in thirty-one sera from patients with sickle cell disease (SCD). The SCD sera were compared with normal sera as a source of the alternative complement pathway factors C3 proactivator (C3PA) and C3PA convertase. These factors are required for formation of the enzymatically active CoVF-C3PA complex which is capable of cleaving C3 and thus initiating generation of the cytolytic C5b-9 complex. CoVF cofactor activity was significantly less than normal in SCD sera as measured in an indirect lysis assay, indicating reduced C3PA or C3PA convertase activity in these sera. Qualitative (immunoelectrophoresis) and quantitative (radial immunodiffusion) measurement of C3PA showed, however, that this protein is normal or elevated in SCD sera. Taken together, the reduced CoVF cofactor activity and normal or elevated C3PA in SCD sera suggests that sera from patients with sickle cell disease have reduced C3PA convertase activity.
Author List
Koethe SM, Casper JT, Rodey GEMESH terms used to index this publication - Major topics in bold
AdolescentAdult
Anemia, Sickle Cell
Animals
Child
Child, Preschool
Complement C3
Complement System Proteins
Enzyme Activation
Esterases
Glycoproteins
Humans
Immunodiffusion
Immunoelectrophoresis
Infant
Sickle Cell Trait
Snakes
Venoms
