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Findings point toward one of first therapies for Lou Gehrig's disease
Date:6/12/2014

nc are important in stabilizing this protein, and can help it remain folded more than 200 years.

"The damage from ALS is happening primarily in the spinal cord and that's also one of the most difficult places in the body to absorb copper," Beckman said. "Copper itself is necessary but can be toxic, so its levels are tightly controlled in the body. The therapy we're working toward delivers copper selectively into the cells in the spinal cord that actually need it. Otherwise, the compound keeps copper inert."

"This is a safe way to deliver a micronutrient like copper exactly where it is needed," Beckman said.

By restoring a proper balance of copper into the brain and spinal cord, scientists believe they are stabilizing the superoxide dismutase in its mature form, while improving the function of mitochondria. This has already extended the lifespan of affected mice by 26 percent, and with continued research the scientists hope to achieve even more extension.

The compound that does this is called copper (ATSM), has been studied for use in some cancer treatments, and is relatively inexpensive to produce.

"In this case, the result was just the opposite of what one might have expected," said Blaine Roberts, lead author on the study and a research fellow at the University of Melbourne, who received his doctorate at OSU working with Beckman.

"The treatment increased the amount of mutant SOD, and by accepted dogma this means the animals should get worse," he said. "But in this case, they got a lot better. This is because we're making a targeted delivery of copper just to the cells that need it.

"This study opens up a previously neglected avenue for new disease therapies, for ALS and other neurodegenerative disease," Roberts said.


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Contact: Joseph Beckman
joe.beckman@oregonstate.edu
541-737-8867
Oregon State University
Source:Eurekalert

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