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Grant supports Lou Gehrigís research (7-24-00)

John Hart, Ph.D., assistant professor of biochemistry at The University of Texas Health Science Center at San Antonio (UTHSC), is edging closer to discovering the cause of the inherited form of amyotrophic lateral sclerosis (ALS). His efforts recently received grant 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. Offspring in families with a history of ALS have a 50 percent chance of inheriting 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 60 different mutant CuZnSOD structures and compare them 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," Dr. Hart said. "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 Graduate School of Biomedical Sciencesí Center for Biomolecular Structure Analysis. He is a participating scientist in the Health Science Centerís Aging Research and Education Center and in the San Antonio Cancer Institute, a partnership of UTHSC and the Cancer Therapy & Research Center.

Contact: Will Sansom