2013 Fiscal Year Final Research Report
Role of lysosome-associated membrane protein LAMP-2 in autophagy
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 |
Section | 一般 |
Research Field |
General anatomy (including Histology/Embryology)
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Research Institution | Juntendo University |
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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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 |
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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Research Products
(10 results)
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[Journal Article] Discovery of a novel type of autophagy targeting RNA2013
Author(s)
Fujiwara Y, Furuta A, Kikuchi H, Aizawa S, Hatanaka Y, Konya C, Uchida K, Yoshimura A, Tamai Y, Wada K, Kabuta T
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Journal Title
Autophagy
Volume: 9
Pages: 403-9
DOI
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[Journal Article] Lysosomal storage and advanced senes- cence in the brain of LAMP-2- deficient Danon disease2013
Author(s)
Furuta A, Wakabayashi K, Haratake J, Kikuchi H, Kabuta T, Mori F, Tokonami F, Katsumi Y, Tanioka F, Uchiyama Y, Nishino I
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Journal Title
Wada K. Acta Neuropathol
Volume: 125
Pages: 459-61
DOI
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[Journal Article] ATP13A2 deficiency induces a decrease in cathepsin D activity, fingerprint-like inclusion body formation, and selective degeneration of dopaminergic neurons2013
Author(s)
Matsui H, Sato F, Sato S, Koike M, Taruno Y, Saiki S, Funayama M, Ito H, Taniguchi Y, Uemura N, Toyoda A, Sakaki Y, Takeda S, Uchiyama Y, Hattori N, Takahashi R
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Journal Title
FEBS Lett
Volume: 587
Pages: 1316-25
DOI
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