(mentions CMT) Mitochondrial Movements May Shed Light On ALS, Other Diseases

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Mitochondrial Movements May Shed Light On ALS, Other Diseases

http://www.mdausa.org/research/070619mito.html

Watching the movements of mitochondria, the energy-producing

miniature organs (organelles) inside cells, under a variety of

conditions has just gotten easier, thanks to a new set of laboratory

tools developed by Jeff Lichtman at Harvard University and

colleagues.

Mitochondria are particularly vital in nerve and muscle cells, which

require a constant energy supply, and abnormalities of mitochondria

play a role in mitochondrial myopathies, Friedreich's ataxia, some

types of Charcot-Marie-Tooth disease and probably other

neuromuscular disorders. Deficient transport of mitochondria up and

down nerve fibers is suspected of contributing to nerve cell loss in

amyotrophic lateral sclerosis (ALS).

In the July issue of Nature Methods, Lichtman describes how the

group developed and bred mice with fluorescent proteins that light

up only the mitochondria. These " MitoMice " allow researchers to take

time-lapse photographs of moving mitochondria in living animals.

Already, they say, they've learned a lot from experimenting with

these mice. For one thing, they now know that, under normal

circumstances, more mitochondria are moving away from the nerve cell

body (anterograde transport) than are moving back toward it

(retrograde transport) at any given time. This suggests, they say,

that some portion of the organelles are destroyed before they head

back to the nerve cell.

The researchers have already begun using the MitoMice to study nerve

injuries, and they say the mice can also be used to study diseases.