The alternative pathway of complement activation may be involved in the renal damage of human anti-glomerular basement membrane disease

Linear deposition of IgG and complement 3 (C3) along glomerular basement membrane (GBM) is generally revealed in the kidneys of human anti-GBM disease. Our recent studies demonstrated the pathogenic role of complement activation in renal damage of this disease. However, the pathways of complement ac...

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Bibliographic Details
Published in:PloS one 2014-03, Vol.9 (3), p.e91250-e91250
Main Authors: Ma, Rui, Cui, Zhao, Hu, Shui-Yi, Jia, Xiao-Yu, Yang, Rui, Zheng, Xin, Ao, Jie, Liu, Gang, Liao, Yun-Hua, Zhao, Ming-Hui
Format: Article
Language:English
Subjects:
Adult
Aged
Alternative pathway
Anti-Glomerular Basement Membrane Disease - metabolism
Anti-Glomerular Basement Membrane Disease - pathology
Basement membranes
Biology and Life Sciences
Biopsy
Bowman's capsule
Classical pathway
College professors
Complement
Complement Activation
Complement C3 - metabolism
Complement component C1q
Complement component C3
Complement factor B
Damage
Deposition
Disease prevention
Female
Fluorescent Antibody Technique
Humans
Immunofluorescence
Immunoglobulin G
Immunoglobulin G - metabolism
Immunohistochemistry
Kidney - metabolism
Kidney - pathology
Kidneys
Lectins
Male
Mannose
Mannose-binding lectin
Medical research
Medicine and Health Sciences
Mesangium
Middle Aged
Patients
Properdin
Rodents
Tissues
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Summary:Linear deposition of IgG and complement 3 (C3) along glomerular basement membrane (GBM) is generally revealed in the kidneys of human anti-GBM disease. Our recent studies demonstrated the pathogenic role of complement activation in renal damage of this disease. However, the pathways of complement activation were still paradoxical. In this study, renal biopsy tissues from 10 patients with anti-GBM disease were used to investigate the pathways of complement activation by detecting the deposition of various complement components, including C1q, factor B, factor P (properdin), mannose-binding lectin (MBL), C3d, C4d and C5b-9, using immunohistochemistry and immunofluorescence. We found that C1q, factor B, properdin, C3d, C4d and C5b-9 were detected in all the glomeruli of our patients, along GBM with a linear and/or granular staining pattern. Furthermore, C1q, factor B and properdin co-localized well with C5b-9. The properdin also co-localized well with C3d. However, the deposition of MBL was diffusive in mesangium, GBM, Bowman's capsule and within crescents and was not co-localized with C5b-9 but partially co-localized with C4d. The intensity of factor B deposition (3.3 vs. 1.2, P
ISSN:1932-6203
1932-6203
DOI:10.1371/journal.pone.0091250