July 7, 2000
Volume XXXIII, No. 27

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ALS researcher receives funding support

Picture of Doctor Hart
Dr. Hart

Dr. P. John Hart, assistant professor in the Department of Biochemistry, is edging closer to discovering the cause of the inherited form of amyotrophic lateral sclerosis (ALS). His efforts recently received grant funding support from the ALS Association and the National Institutes of Health (NIH).

Dr. Hart will receive $75,000 in funding from the ALS Association over the next three years and $775,000 from NIH for the next five years.

ALS is a progressive fatal neuro-degenerative disorder also known as Lou Gehrig's disease. It affects an estimated 30,000 Americans.

The most common form of ALS is the "sporadic" version, which accounts for about 90 percent of all cases and may affect anyone at any time. Dr. Hart is concentrating his research on the inherited form of ALS, which accounts for the remaining percentage of people with ALS. In families with a history of ALS, there is a 50 percent chance each offspring will inherit the gene mutation to develop the disorder.

Dr. Hart is looking at mutations in a protein called copper-zinc superoxide dismutase (CuZnSOD) that may contribute to the death of motor neurons. Using X-ray crystallography and analytical ultracentrifugation, Dr. Hart's group hopes to view approximately 60 different mutant CuZnSOD structures and to compare those to the normal structure. Dr. Hart also is researching the structures of another group of proteins called copper chaperones that work in concert with superoxide dismutase.

The hoped-for result of Dr. Hart's research will be a drug therapy that interacts with the chemistry of the mutated proteins to prevent or slow the degeneration of motor neurons in ALS patients.

"Any information we can get immediately is a benefit," said Dr. Hart. "The identification of mutations in this class of proteins represents the first time that we have a real handle on something to look at that causes ALS."

Dr. Hart works in the biochemistry department's Center for Biomolecular Structure Analysis, and is a member of the Aging Research and Education Center as well as the San Antonio Cancer Institute.