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Nat Rev Neurosci. 2008 Jul;9(7):505-18. doi: 10.1038/nrn2417.

Mitochondrial fragmentation in neurodegeneration.

Author information

1
Burnett School of Biomedical Sciences, College of Medicine, University of Central Florida, 4000 Central Florida Boulevard, Orlando, Florida 32816, USA.

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.

PMID:
18568013
PMCID:
PMC2711514
DOI:
10.1038/nrn2417
[Indexed for MEDLINE]
Free PMC Article

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