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The cause of Lou Gehrigs Disease (amyotrophic lateral sclerosis, or ALS) has remained elusive since it brought down one of baseballs greatest players 60 years ago.
ALS starts when good proteins go bad, says Wilfredo Freddie Colón, assistant professor of chemistry. Understanding just why they go bad is a necessary first step toward developing medicines that will help ALS patients live with a manageable disease instead of a death sentence.
If we know what the pathological mechanism is, we could devise drugs to block it, Colón says. Just knowing which protein is different is not enough. We need to do the biochemistry at the molecular level to understand what these mutations are doing to the protein.
The Rensselaer biochemist recently earned a $1 million, four-year grant from the National Institutes of Health (NIH) to support his study of the hereditary version of the disease, called familial ALS (FALS). He is attempting to understand why mutants of the enzyme superoxide dismutase (SOD1) fail and misfunction in FALS.
On average, FALS strikes people at around 47 years of age, says Colón. Most patients die within two to five years. But some patients, whose proteins exhibit a different kind of mutation, experience a very slow progression and can survive for as long as 18 years, he says.
The existence of the enzyme mutants associated with this milder form of FALS makes it an intriguing biophysical and biochemical marker. Unlocking the mystery of the pathogenic causes for familial ALS could also play an important role in better understanding other neurogenerative diseases, such as Parkinsons and Alzheimers.
According to the NIH, ALS is one of the most common neuromuscular diseases worldwide. There is no known cure for the disease that eventually paralyzes and kills its victims.
|Rensselaer Magazine: June 2002|
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