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Leaky blood vessels open up nerve cells to toxic assault in Lou Gehrig's disease
Date:4/7/2008

Leaky blood vessels that lose their ability to protect the spinal cord from toxins may play a role in the development of amyotrophic lateral sclerosis, better known as ALS or Lou Gehrigs disease, according to research published in the April issue of Nature Neuroscience.

The results mark the first time that scientists have witnessed molecular changes occurring long before key nerve cells start dying. The unexpected finding opens up a new front in studies of ALS, a disease in which motor neurons in the spinal cord die off for unknown reasons, resulting in dramatically weakened muscles. Patients lose their strength, their ability to move or swallow, and eventually lose their ability even to breathe. Most patients live only a few years after diagnosis.

We believe these changes contribute to or possibly initiate the onset of ALS, said lead author Berislav Zlokovic, M.D., Ph.D., of the University of Rochester Medical Center. Its clear that these changes occur before the loss of neurons, and its well known that the types of changes we are seeing certainly injure or kill these types of cells, which are extremely sensitive to their biochemical environment.

The results, discovered by studying mutant mice that have an inherited form of the disease, were made by a collaboration of neuroscientists from the University of Rochester Medical Center working together with a team of ALS experts from the University of California at San Diego. Zlokovic, a pioneer in learning how the bodys vascular system plays a role in neurodegenerative diseases like Alzheimers disease and ALS, led the team, and the first author is post-doctoral researcher Zhihui Zhong, Ph.D.

While its unlikely the new findings will help ALS patients immediately, the results open up a new and unexpected way to think about the disease. Zlokovics team is currently testing in the laboratory a compound that may help seal up leaky vessels and protect the neurons targeted by ALS.


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Contact: Tom Rickey
tom_rickey@urmc.rochester.edu
585-275-7954
University of Rochester Medical Center
Source:Eurekalert

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