| 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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| 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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