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Mitochondrial fragmentation in neurodegeneration.

ABSTRACT: Mitochondria are remarkably dynamic organelles that migrate, divide and fuse. Cycles of mitochondrial fission and fusion ensure metabolite and mitochondrial DNA mixing and dictate organelle shape, number and bioenergetic functionality. There is mounting evidence that mitochondrial dysfunction is an early and causal event in neurodegeneration. Mutations in the mitochondrial fusion GTPases mitofusin 2 and optic atrophy 1, neurotoxins and oxidative stress all disrupt the cable-like morphology of functional mitochondria. This results in impaired bioenergetics and mitochondrial migration, and can trigger neurodegeneration. These findings suggest potential new treatment avenues for neurodegenerative diseases.

SUBMITTER: Knott AB

PROVIDER: S-EPMC2711514 | biostudies-literature | 2008 Jul

REPOSITORIES: biostudies-literature

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Mitochondrial fragmentation in neurodegeneration.

Knott Andrew B AB Perkins Guy G Schwarzenbacher Robert R Bossy-Wetzel Ella E

Nature reviews. Neuroscience 20080701 7

Mitochondria are remarkably dynamic organelles that migrate, divide and fuse. Cycles of mitochondrial fission and fusion ensure metabolite and mitochondrial DNA mixing and dictate organelle shape, number and bioenergetic functionality. There is mounting evidence that mitochondrial dysfunction is an early and causal event in neurodegeneration. Mutations in the mitochondrial fusion GTPases mitofusin 2 and optic atrophy 1, neurotoxins and oxidative stress all disrupt the cable-like morphology of fu ...[more]

PMID: 18568013

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