Document Detail

Glomerular deposition of the complement C4 isotypes C4A and C4B in glomeruonephritis.
MedLine Citation:
PMID:  8671963     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Complement C4 is a component of the classical complement pathway, which is a major mediator of inflammation in many forms of glomerulonephritis. The two isoforms of C4-C4A and C4B-differ in their physicochemical and functional properties. METHODS: The glomerular deposition of C4A and C4B was investigated in 39 cases of glomerulonephritis with classical pathway activation using isotype-specific monoclonal antibodies 99H7 (C4A) and 1288 (C4B) and indirect immunofluorescence. Complement C4 phenotypes of all patients were determined by agarose gel electrophoresis and immunoprecipitation. RESULTS: Three biopsies contained only the isotype C4B. C4 phenotyping revealed complete C4A deficiency in these three patients. Both isotypes C4A and C4B were detected in 36 biopsies. In 19 (53%) thereof staining for both isotypes was identical. In the remaining 17 (47%), staining intensity of C4A predominated over C4B. The distribution of these two staining patterns did not differ between membranous glomerulonephritis and lupus nephritis. They were also independent of C4A and C4B allotypes including the presence or absence of null alleles at either gene locus. In no case was C4B staining stronger than C4A staining. Serum creatinine and proteinuria did not differ between patients with identical and C4A-dominant C4 deposition. CONCLUSIONS: The most likely but still hypothetical explanation for predominance in glomerular deposition of C4A over C4B in many cases of immune complex-mediated glomerulonephritis is the greater affinity of C4A to protein-containing immune complexes as compared to C4B.
K Lhotta; A Schlogl; F Kronenberg; M Joannidis; P Konig
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association     Volume:  11     ISSN:  0931-0509     ISO Abbreviation:  Nephrol. Dial. Transplant.     Publication Date:  1996 Jun 
Date Detail:
Created Date:  1996-12-17     Completed Date:  1996-12-17     Revised Date:  2010-03-24    
Medline Journal Info:
Nlm Unique ID:  8706402     Medline TA:  Nephrol Dial Transplant     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  1024-8     Citation Subset:  IM    
Department of Internal Medicine, Innsbruck University Hospital and Instute of Medical Biology and Human Genetics, University of Innsbruck, Austria.
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MeSH Terms
Complement C4 / genetics
Complement C4a / metabolism*
Complement C4b / metabolism*
Fluorescent Antibody Technique
Glomerulonephritis / genetics,  physiopathology
Kidney / physiopathology
Kidney Glomerulus / metabolism*
Middle Aged
Polymorphism, Genetic
Staining and Labeling
Reg. No./Substance:
0/Complement C4; 80295-49-4/Complement C4a; 80295-50-7/Complement C4b

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