Relative importance of C3b inactivator and beta 1H globulin in the modulation of the properdin amplification loop in systemic lupus erythematosus.
Serum concentrations of C4, C3, factor B (B), properdin (P), C3b inactivator (C3bINA) and beta 1H globulin have been measured by radial immunodiffusion in sixty-two samples from thirteen patients with systemic lupus erythematosus (SLE). Significant reductions in the mean serum concentrations of C4 (classical pathway) B and P (alternative pathway) and C3 were found. In addition, the mean level of the control protein beta 1H, but not C3bINA, was reduced. Sera from thirteen patients taking during disease exacerbation (low C3) showed significantly lower levels of both C3bINA and beta 1H than sera taken from the same thirteen patients during disease remission (high C3). Serum concentrations of C3bINA correlated with B (P less than 0.005) but not C4, C3 or P, whereas levels of beta 1H correlated with C4 (P less than 0.01), B (P less than 0.005) and properdin (P less than 0.01). Serial measurements of the serum concentrations of C3bINA and beta 1H showed that levels of these protein fell during exacerbation, and such falls were more closely associated with diseases in the serum levels of the alternative pathways proteins than C4. It is concluded from these observations that serum concentrations of the control proteins C3bINA and beta 1H, especially the latter, control the extent of turnover of the alternative pathway in SLE. Metabolic studies are required to determine the causes of the decreased serum concentrations of these control proteins.
D003167:Complement Activation; D003176:Complement C3 / Q000032:analysis; D003180:Complement C3b Inactivator Proteins / Q000097:blood*; D003181:Complement C4 / Q000032:analysis; D011415:Complement Factor B / Q000032:analysis; D003170:Complement Pathway, Alternative; D006801:Humans; D005779:Immunodiffusion; D008180:Lupus Erythematosus, Systemic / Q000276:immunology*; D011414:Properdin / Q000097:blood; D012075:Remission, Spontaneous; D013997:Time Factors
D003176:Complement C3; D003180:Complement C3b Inactivator Proteins; D003181:Complement C4; D011414:Properdin; D011415:Complement Factor B