Demonstration of beta 1H globulin in pemphigus.
beta 1H globulin is a plasma protein which regulates the biologic activities of the major fragment of the 3rd complement component, C3b. The role of beta 1H globulin in pemphigus was investigated using immunofluorescence in the present study. Lesional skin biopsies from patients with confirmed pemphigus demonstrated in-vivo deposition of beta 1H in addition to C3 in all of four biopsies. Eight serum samples containing C3 fixing intercellular antibodies were then tested for the capacity to fix beta 1H and other complement components. All eight pemphigus sera showed fixation of beta 1H to the intercellular areas of normal human skin. C1q and C4 fixation by pemphigus sera was also demonstrated in 7 of 8 sera, respectively. The experiment using C2-deficient serum indicated that the fixation of beta 1H by intercellular antibodies requires the activation of the classical complement pathway. These data suggested that beta 1H, a co-factor of C3b inactivator, plays a role in the in-vivo regulation of complement activity and supplies additional evidence for the participation of complement system in the pathogenesis of pemphigus.
D000918:Antibody Specificity; D003179:Complement C3b / Q000032:analysis / Q000276:immunology; D003180:Complement C3b Inactivator Proteins / Q000032:analysis / Q000276:immunology*; D017242:Complement Factor H; D003168:Complement Fixation Tests; D003171:Complement Pathway, Classical; D005455:Fluorescent Antibody Technique; D006801:Humans; D010392:Pemphigus / Q000276:immunology* / Q000473:pathology
C493495:CFH protein, human; D003180:Complement C3b Inactivator Proteins; D003179:Complement C3b; D017242:Complement Factor H