| Budget Amount *help |
¥4,940,000 (Direct Cost: ¥3,800,000、Indirect Cost: ¥1,140,000)
Fiscal Year 2013: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2012: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2011: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
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| Research Abstract |
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease that is a leading cause of end-stage renal disease.Isolated autosomal dominant polycystic liver disease (ADPLD) is genetically distinct from PLD associated with ADPKD. ADPLD is caused by mutations in SEC63 gene. The mechanism of cyst development is not still clear. The aim of this project is to elucidate mechanisms of cyst development. We revealed that there is a dose-response relationship between cystic dilation and levels of functional polycystin-1 in Sec63 conditional knockout mice. In addition, cell proliferation is one of the main factor of cyst progression in Pkd1 deficient model. However, we found that cell proliferation accelerates cyst growth in the early stage Sec63 deicient model, but the rate of apoptosis is increased at a late stage. These results suggest that the mechanism of cyst progression is different between Sec63 deficient mice pkd1 deficient mice.
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