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- W2561248436 abstract "In the nervous system, many axons are ensheathed by myelin, and dysmyelination disorders are congenital defects in myelin which often lead to loss of myelin called demyelination and axonal degeneration. Although mitochondria are important for axonal functions and survival, mechanisms and roles of mitochondrial alterations in dysmyelinated axons remained elusive. In this study, mitochondrial alterations in the dysmyelinated axons were studied in PLP transgenic mice with overexpression of proteolipid proteins (PLPtg) [ 1 ]. With serialblock-face scanning electron microscopy (SBF-SEM), the volume ratio of mitochondria (mitochondrial volume /axonal volume) at 1 month old (m.o.) is similar in myelinated axons of WT and PLPtg. The volume ratio is significantly increased in demyelinated axons but not in myelinated axons of 3 m.o. PLPtg, primarily by increasing the mitochondrial number. By contrast, at 5m.o. when most optic nerve axons are demyelinated in PLPtg, volume of each mitochondrion but not mitochondrial number is dramatically increased in the demyelinated axons, whereas cristae morphology of the large mitochondria was well maintained. Finally, we established organotypic cerebellar slice cultures, and observed robust myelination in WT slices and gradual myelin loss in PLPtg slices during maintenance. These results demonstrate acute mitochondrial accumulation and chronic mitochondrial enlargement upon myelin loss in dysmyelinated axons, and suggest that the enlargement is due to extensive mitochondrial fusion. The results also suggest that slice cultures can be useful to observe mitochondrial behaviour under dysmyelination in vitro." @default.
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- W2561248436 date "2015-11-01" @default.
- W2561248436 modified "2023-10-16" @default.
- W2561248436 title "C1-P-02Analyses of mitochondrial changes in dysmyelinated axons" @default.
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- W2561248436 doi "https://doi.org/10.1093/jmicro/dfv296" @default.
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