A simple immunofixation test for induced C3 degradation in disease states (including C3 nephritic factor) and its correlation with kidney biopsy.
Complement dysregulation from an uncontrolled activation of the alternate pathway can be mediated by C3 Nephritic Factor and results in C3 glomerulopathy. Identification of C3 degradation products C3c and C3d in patient serum provides evidence of uncontrolled complement activation. It is possible to detect C3c and C3d in patient serum by an immunofixation assay which induces in vitro C3 degradation. The clinical performance of the immunofixation assay has been assessed by comparing the assay results with findings from immunostaining of kidney biopsies. The immunofixation assay is a simple and reliable technique for detection of C3 degradation on a widely available platform and can be used to provide corroborative evidence of acquired complement dysregulation in patients with C3 glomerulopathy.
Department of Immunology and Immunogenetics, Pathology Building, Southmead Hospital, Westbury-on-Trym, Bristol BS10 5NB, United Kingdom. Electronic address: esha.sarkar@nbt.nhs.uk.
Esha
E
Sarkar
Department of Immunology and Immunogenetics, Pathology Building, Southmead Hospital, Westbury-on-Trym, Bristol BS10 5NB, United Kingdom. Electronic address: ellen.roberts@nbt.nhs.uk.
Ellen G
EG
Roberts
Department of Immunology and Immunogenetics, Pathology Building, Southmead Hospital, Westbury-on-Trym, Bristol BS10 5NB, United Kingdom. Electronic address: michael.wallage@nbt.nhs.uk.
Michael J
MJ
Wallage
Department of Immunology and Immunogenetics, Pathology Building, Southmead Hospital, Westbury-on-Trym, Bristol BS10 5NB, United Kingdom. Electronic address: joe.unsworth@nbt.nhs.uk.
David J
DJ
Unsworth
Department of Immunology and Immunogenetics, Pathology Building, Southmead Hospital, Westbury-on-Trym, Bristol BS10 5NB, United Kingdom. Electronic address: robert.lock@nbt.nhs.uk.
Robert J
RJ
Lock
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003167:Complement Activation; D003178:Complement C3 Nephritic Factor / Q000032:analysis*; D015932:Complement C3c / Q000032:analysis*; D015933:Complement C3d / Q000032:analysis*; D003170:Complement Pathway, Alternative; D005260:Female; D005921:Glomerulonephritis / Q000097:blood / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D007158:Immunologic Techniques; D007223:Infant; D007668:Kidney / Q000276:immunology* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D055815:Young Adult
D015415:Biomarkers; D003178:Complement C3 Nephritic Factor; D015932:Complement C3c; D015933:Complement C3d
C3 Nephritic Factor; C3 degradation; C3 glomerulopathy; Complement dysregulation; Immunofixation assay; Kidney biopsy