July 18, 1997

Cell-Death Gene Linked to ALS

The ICE cell-death gene has been linked to amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. Researchers at Massachusetts General Hospital showed that a mutation in ICE was able to slow the progression of ALS-like disease in mice. ALS in humans is a degenerative disorder of the brain and spinal cord that leads to paralysis of muscles and death. The results were published in the July 3 issue of Nature.

The team, led by Junying Yuan, now an associate professor of cell biology at HMS, discovered ICE's involvement by breeding transgenic mice with two mutated genes. Mice with a mutation in the gene for superoxide dismutase (SOD1), were crossed with mice with a mutation in the ICE (interleukin-1 beta-converting enzyme) gene. The researchers found that mice with both mutations developed the disease at the same time as the single, SOD1-deficient mice. But mice with both mutations lived longer after getting the disease--27 versus 12 days.

Now that researchers know that ICE is involved in ALS, they can work to develop a drug that inhibits ICE and slows the progression of ALS.

Other Harvard authors of the paper were Robert M. Friedlander, Robert H. Brown Jr., Valeria Gagliardini, and Joy Wang.