2015 Fiscal Year Final Research Report
Involvement of the complemet factor B in the etiology of membranous proliferative glomerulonephritis
Project/Area Number |
25860873
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Research Category |
Grant-in-Aid for Young Scientists (B)
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Allocation Type | Multi-year Fund |
Research Field |
Pediatrics
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Research Institution | University of Miyazaki |
Principal Investigator |
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Project Period (FY) |
2013-04-01 – 2016-03-31
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Keywords | 膜性増殖性糸球体腎炎 / 補体 / 非典型溶血性尿毒症症候群 |
Outline of Final Research Achievements |
We reveal that CFB p.S367R has gain-of-function effects in CFB from the finding including structure-function relationship of CFB p.S367R and elevated soluble C5b-9 levels which indicate continuous activity of the complement alternative pathway. This is the first report that demonstrates the involvement of CFB in the etiology of MPGN. However, culturing a large quantity of CFB p.S367R was unsuccessful. Evaluation of soluble C5b-9 levels in patients with thrombotic microangiopathy(TMA) revealed complement activations contribute not only in the etiology of atypical hemolytic uremic syndrome, but also in shiga toxin producing E.coli-associated HUS and secondary TMA.
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Free Research Field |
小児腎疾患
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