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(Ivanhoe Newswire) -- New light is being shed on how a specific gene mutation produces the symptoms of Lou Gehrig's disease. Knowing how this gene mutation works may mean a better way to predict patient longevity.
Amyotrophic Lateral Sclerosis (ALS) -- or Lou Gehrig's disease -- is a fatal neurodegenerative disease with no known treatment. Until now, it has not been known how a specific gene mutation can cause the condition.
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Researchers at Brandies and Harvard Universities have discovered that they can predict ALS patient longevity based on two properties of the protein SOD1. Both the stickiness of SOD1 and its decreased stability account for 69 percent of patient survival data. This provides evidence that SOD1 protein stability and its aggregation propensity are the main toxic causes of ALS.
SOD1 has been shown to be mutated in at least 119 different ways in different ALS patients. Some mutations have a more dramatic effect than others, but it's now believed that it's the sticking together of SOD1 that is toxic.
ALS can occur spontaneously in people with no family history of the condition, but about 10 percent of cases run in families. In about 20 percent of cases in that subset the underlying mutation is a change in the gene for SOD1.
SOURCE: PLoS Biology, published online July 28, 2008.
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