| Project/Area Number |
23590244
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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 |
General anatomy (including Histology/Embryology)
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| Research Institution | Juntendo University |
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Principal Investigator |
FURUTA Akiko 順天堂大学, 医学(系)研究科(研究院), 非常勤助教 (50229118)
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| Co-Investigator(Kenkyū-buntansha) |
KOIKE Masato 順天堂大学, 医学部, 准教授 (80347210)
SASAKI Mitsuho 順天堂大学, 医学部, 助教 (20432536)
SUNABORI Takehiko 順天堂大学, 医学部, 助教 (00407115)
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| Project Period (FY) |
2011 – 2013
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| Project Status |
Completed (Fiscal Year 2013)
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| Budget Amount *help |
¥5,200,000 (Direct Cost: ¥4,000,000、Indirect Cost: ¥1,200,000)
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: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
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| Keywords | リソソーム / オートファジー / ダノン病 / リソソーム蓄積病 / 肥大型心筋症 / LAMP-2 / 膜タンパク質 / シャペロン介在性オートファジー / リソソーム膜タンパク質 / 神経変性疾患 / 心筋症 |
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| Research Abstract |
Lysosome-associated membrane protein-2 (LAMP-2) is the gene responsible for Danon disease. To elucidate the function of LAMP-2, we have investigated the histopathological changes in LAMP-2-deficient mice. Although a large amount of autophagic vacuoles in parenchymal cells of the visceral organs, these findings were rarely detected in the brain tissue. By ultrastructural studies, various-shaped accumulations were found in the neuronal cells of LAMP-2-deficient brains. These pathological features indicate lysosomal storage disease. We also examined the brain lesions from the neonatal hypoxia ischemia and MCA occlusion in the LAMP-2-deficient mice. Pathological examination revealed that extensive tissue damage with altered calcium-related signaling was found in the models of the LAMP-2-deficient mice. Thus, LAMP-2 in the CNS has a possible role in degradation of the various macromolecules in lysosomes and an additional function concerning tissue repair during the pathological processes.
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