| Budget Amount *help |
¥2,600,000 (Direct Cost: ¥2,000,000、Indirect Cost: ¥600,000)
Fiscal Year 2014: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
Fiscal Year 2013: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
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| 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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