2010 Fiscal Year Final Research Report
Understanding the mechanisms underlying the pathogenesis of Hirschsprung disease
| Project/Area Number |
20590204
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
General anatomy (including Histology/Embryology)
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| Research Institution | The Institute of Physical and Chemical Research |
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Principal Investigator |
UESAKA Toshihiro The Institute of Physical and Chemical Research, 神経分化・再生研究チーム, 研究員 (90304451)
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| Project Period (FY) |
2008 – 2010
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| Keywords | ヒルシュスプルング病 / Ret / 細胞死 / 神経堤細胞 / Bcl-xL / 腸管ニューロン |
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| Research Abstract |
RET tyrosine kinase is required for the development of the enteric nervous system (ENS). Hypomorphic RET alleles cause intestinal aganglionosis [Hirschsprung disease (HSCR)]. We have shown that elevated expression of Bcl-xL inhibits ENS precursor death in both Ret-null and hypomorphic states. Moreover, the prevention of cell death allows morphologically and functionally normal ENS formation in Ret-hypomorphic mice. These results indicate that ENS precursor death is a principal cause of intestinal aganglionosis in a Ret hypomorphic state, and suggest that the inhibition of cell death is a route to the prevention of HSCR.
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Research Products
(9 results)
