Molecular mechanism and pathological analysis of mitochondrial chaperon protein p32
| Project/Area Number |
24590387
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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 |
Pathological medical chemistry
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| Research Institution | Kyushu University |
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Principal Investigator |
UCHIUMI Takeshi 九州大学, 医学(系)研究科(研究院), 准教授 (80253798)
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| Project Period (FY) |
2012-04-01 – 2015-03-31
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| Project Status |
Completed (Fiscal Year 2014)
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| Budget Amount *help |
¥5,460,000 (Direct Cost: ¥4,200,000、Indirect Cost: ¥1,260,000)
Fiscal Year 2014: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2013: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
Fiscal Year 2012: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
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| Keywords | ミトコンドリア / p32 / 翻訳 / ノックアウトマウス / ストレス / 神経変性疾患 / 酸化的リン酸化 |
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| Outline of Final Research Achievements |
The mitochondrial matrix protein p32/ C1qBP functions as an essential RNA and protein chaperon in mitochondrial translation, and is indispensable for embryonic development. To investigate the functional role of p32 in an organism, we deleted p32 selectively in neurons and glia (p32NesKO mice). The neuron-specific p32KO mice showed grow retardation and death by ~8 weeks. P32 KO mice causes significant axonal degeneration and demyelination in mid brain brainstem are particularly involved. We show that disruption of glial mitochondria activates an adaptive integrated stress response. We also observed that severe protein expression, dysfunction of the mitochondrial respiratory chain, and reduced oxygen consumption. Our data suggest that oligodendrocyte dysfunction and axon degeneration may be a contributor to mitochondria-related leukoencephalopathy.
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Report
(4 results)
Research Products
(12 results)
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[Journal Article] Tor and the Sin3-Rpd3 complex regulate expression of the mitophagy receptor protein Atg32 in yeast.2014
Author(s)
Aihara M, Jin X, Kurihara Y, Yoshida Y, Matsushima Y, Oku M, Hirota Y, Saigusa T, Aoki Y, Uchiumi T, Yamamoto T, Sakai Y, Kang D, Kanki T.
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Journal Title
Journal of Cell Science
Volume: 127
Pages: 3184-96
DOI
Related Report
Peer Reviewed / Open Access
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[Journal Article] Protein instability and functional defects caused by mutations of dihydro-orotate dehydrogenase in Miller syndrome patients.2012
Author(s)
Fang J, Uchiumi T, Yagi M, Matsumoto S, Amamoto R, Saito T, Takazaki S, Kanki T, Yamaza H, Nonaka K, Kang D.
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Journal Title
Biosci Rep
Volume: 32
Issue: 6
Pages: 631-639
DOI
Related Report
Peer Reviewed
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