First published online May 28, 2004
Journal of Cell Science 117, 1305e (2004)
© The Company of Biologists Limited
Mitochondrial DNA dynamics
Mutations in mitochondrial DNA (mtDNA) cause several neuromuscular disorders. Frequently these mutations are heteroplasmic i.e. mutant and wild-type molecules coexist within the same cell. It is therefore important to define the mechanisms that govern the organization, mobility and segregation of mtDNA in human cells. Using antibodies directed against DNA, mtDNA-binding proteins and other mitochondrial proteins, Manuel Rojo and co-workers have analysed the localization and dynamics of mtDNA in cultured human cells (see p. 2653). They observe that the cell's mitochondrial network possesses several hundred nucleoids, each of which contains 2-8 mtDNA molecules along with associated proteins. They then show that these exhibit a mobility similar to that of soluble mitochondrial matrix proteins and can spread efficiently into mitochondria lacking mtDNA following cell fusion. By contrast, such fusion-mediated transfer is more limited if the recipient mitochondria already contain mtDNA. This reduced mobility leads to spatial segregation of different mtDNA molecules and has significant implications for individuals who have a mixture of mutant and wild-type mtDNA molecules.

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Related articles in JCS:
- Organization and dynamics of human mitochondrial DNA
- Frédéric Legros, Florence Malka, Paule Frachon, Anne Lombès, and Manuel Rojo
JCS 2004 117: 2653-2662.
[Abstract]
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