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Hope for Neuromuscular Disease

Ivanhoe Newswire


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(Ivanhoe Newswire) -- People suffering from neuromuscular disorders may one day benefit from a study conducted in mice.

Researchers found increasing the number of mitochondria in each cell caused mice with these types of disorders to function better and live longer.

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The University of Miami School of Medicine investigators achieved these goals through two different strategies. In one, mice were treated with genetic modifications. In the other, they gave the mice a drug called bezafibrate thats already been approved by the U.S. Food and Drug Administration for the treatment of metabolic conditions. The drug activates the same substance targeted by the genetic treatment.

In some of the mice, the number of mitochondria increased by four or five times following the treatment.

While mice with impaired mitochondria usually only live three or four months, mice that received the genetic solution lived about a year. Mice treated with bezafibrate lived about six months. Both sets of mice functioned better when compared with untreated mice. The genetically treated mice, for example, didnt begin falling on the treadmill test until they were around seven months of age. Untreated mice began falling around three months. The bezafibrate treated mice maintained their performance on the treadmill until they were about six months old.

The investigators note bezafibrate causes some side effects that will have to be considered before the treatment is used in humans.

SOURCE: Cell Metabolism, published online September 2, 2008

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Last updated 9/8/2008

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