Clinical evaluation and characterization of a unique C3 breakdown factor detected in a patient with acute glomerulonephritis.
A unique factor causing breakdown of the third component of complement (C3) was detected in a patient with acute glomerulonephritis (AGN). The activity was detected by conversion of C3 in a mixture with normal human serum using crossed immunoelectrophoresis. This factor capable of C3 breakdown was not blocked in the presence of ethylenediaminetetraacetic acid (EDTA), heat-pretreatment (56 degrees C, 30 min) und dialysis against 0.1 M phenylmethylsulfonyl fluoride (PMSF). The factor was isolated and characterized by immunochemical and physicochemical techniques. It had beta-mobility on electrophoresis and was separated at a lighter sedimentation coefficient than 7S by sucrose density gradient ultracentrifugation. The activity was absorbed on a DEAE-cellulose column and was eluted under definite conditions of relative salt concentration. These data appear to suggest that this factor is distinct from C3 nephritic factor (C3NeF), immunoglobulins, immune complexes, and other C3 convertases previously described. Moreover, it is clear that the factor was mainly responsible for the C3 activation observed in the patient with acute glomerulonephritis.
D000208:Acute Disease; D000328:Adult; D002500:Centrifugation, Isopycnic; D002848:Chromatography, DEAE-Cellulose; D003176:Complement C3; D003169:Complement Inactivator Proteins / Q000302:isolation & purification / Q000378:metabolism*; D003165:Complement System Proteins / Q000378:metabolism; D004591:Electrophoresis, Polyacrylamide Gel; D005260:Female; D005921:Glomerulonephritis / Q000276:immunology*; D006801:Humans; D007123:Immunoelectrophoresis, Two-Dimensional
D003176:Complement C3; D003169:Complement Inactivator Proteins; D003165:Complement System Proteins