2013 Fiscal Year Final Research Report
Analysis of mechanisms of cyst development in Sec63 conditional knockout mice.
| Project/Area Number |
23591175
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Kidney internal medicine
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| Research Institution | Hokkaido University |
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Principal Investigator |
NISHIO Saori 北海道大学, 大学病院, 助教 (90463736)
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| Project Period (FY) |
2011 – 2013
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| Keywords | 多発性肝嚢胞 / Sec63 / 多発性嚢胞腎 / 細胞増殖 / アポトーシス / pkd1 |
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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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