期刊
KIDNEY INTERNATIONAL
卷 84, 期 3, 页码 441-450出版社
ELSEVIER SCIENCE INC
DOI: 10.1038/ki.2013.134
关键词
alternative pathway; C3 glomerulopathy; complement activation; hemolytic uremic syndrome; ischemia-reperfusion injury
资金
- JSPS [24591189, 24390213]
- Ministry of Health, Labour and Welfare of Japan
- Grants-in-Aid for Scientific Research [24591189, 24390213] Funding Source: KAKEN
The complement system functions as a part of the innate immune system. Inappropriate activation of the complement pathways has a deleterious effect on kidneys. Recent advances in complement research have provided new insights into the pathogenesis of glomerular and tubulointerstitial injury associated with complement activation. A new disease entity termed 'C3 glomerulopathy' has recently been proposed and is characterized by isolated C3 deposition in glomeruli without positive staining for immunoglobulins. Genetic and functional studies have demonstrated that several different mutations and disease variants, as well as the generation of autoantibodies, are potentially associated with its pathogenesis. The data from comprehensive analyses suggest that complement dysregulation can also be associated with hemolytic uremic syndrome and more common glomerular diseases, such as IgA nephropathy and diabetic kidney disease. In addition, animal studies utilizing genetically modified mice have begun to elucidate the molecular pathomechanisms associated with the complement system. From a diagnostic point of view, a noninvasive, MRI-based method for detecting C3 has recently been developed to serve as a novel tool for diagnosing complement-mediated kidney diseases. While novel therapeutic tools related to complement regulation are emerging, studies evaluating the precise roles of the complement system in kidney diseases will still be useful for developing new therapeutic approaches.
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