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http://www.abcnews.com/sections/living/DailyNews/gehrigs0303.html March 3, 1998 WASHINGTON (Reuters) -- Researchers said Tuesday they had tracked down a gene responsible for a form of the muscle-wasting disease amyotrophic lateral sclerosis, also known as Lou Gehrig's disease. The gene is somewhere on chromosome 9, the scientists at Johns Hopkins University and the University of Pennsylvania said. The next step is to map and identify the gene itself, they reported in the American Journal of Human Genetics. "These results bring us a major step closer to isolating the gene itself," Dr. David Cornblath, a neurology professor at Johns Hopkins, said in a statement. The researchers studied a Maryland family, the Mattinglys, who have suffered ALS through several generations. "When we do that and clone it, we can find what it does. That should help us treat not only the disease that afflicts the Mattinglys, but also similar neurodegenerative disorders that affect a broader range of patients," Cornblath said. ALS includes a range of diseases that kill the motor nerve cells that control muscles. The muscles waste away and become paralyzed. Baseball player Lou Gehrig died of ALS and physicist Stephen Hawking suffers from a form known in his native Britain as motor neuron disease. About 30,000 Americans suffer from ALS, which usually kills within five years as the muscles that control breathing waste away and paralysis sets in. The version that affects the Mattinglys usually starts to show up in childhood and is never fatal, although victims are badly disabled. Two genes already identified on chromosome 9 are potential suspects, Dr. Phillip Chance, formerly of Children's Hospital of Philadelphia and now at the University of Washington at Seattle. They include genes that create a calcium channel on the surface of nerve cells and are involved in programmed cell death, known as apoptosis. "However, these are only two genes from a large segment of chromosome 9," Chance said. "Geneticists haven't fully characterized this region, so there are a number of other genes we will have to study." Other research has also linked ALS with this programmed cell death. This process usually has a housekeeping function, making sure the body gets rid of old or damaged cells. Scientists in the United States and Switzerland found last July that mice with symptoms of the disease could live longer if they were made to produce large amounts of a protein, bcl-2, involved in apoptosis. Copyright © 1998 Reuters Judith Richards [log in to unmask]