Lipid abnormalities linked to Lou Gehrigs disease

Abnormal accumulation of two common lipids in motor nerve cells could play a critical role in the development of amyotrophic lateral sclerosis (ALS), according to investigators at the National Institute on Aging (NIA) in Baltimore. The finding could help scientists develop drugs and other treatments that might one day slow or arrest the disease's progression.

"ALS is a terrible disease in which a fully functioning mind is trapped inside a body that is becoming progressively paralyzed. At the present time, nothing can be done for ALS, but we hope this newly established link between lipid regulation and the disease will hasten the development of new treatments," said Mark Mattson, Ph.D., lead author of the study* and chief of the NIA Laboratory of Neurosciences. Lipids are the building blocks of fats. The study is available online at the Annals of Neurology website, http://www3.interscience.wiley.com/ and will be published in the journal's September 2002 issue.

Also called Lou Gehrig's disease, ALS is a progressive, fatal neurological disease affecting as many as 20,000 Americans, with 5,000 new cases occurring in the United States each year. Patients usually die within five years of diagnosis. ALS occurs when specific nerve cells in the brain and spinal cord that control voluntary movement gradually degenerate. The loss of these motor neurons causes the muscles under their control to weaken and waste away, leading to paralysis. In some instances, the disease is inherited, but in most cases the cause is unknown.

But NIA scientists unearthed several new clues through a complex, multi-step investigation. After comparing spinal cord tissue extracted from people who had ALS with those who didn't, the investigators discovered that levels of ceramides, cholesterol esters and several other lipids were significantly elevated in the spinal cords of people with ALS.

To test whether these elevated levels of ce

Contact: Doug Dollemore
NIH/National Institute on Aging

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