Duchenne Muscular Dystrophy research focuses on early mechanisms

Main Category: Muscular Dystrophy / ALS
Article Date: 12 Nov 2004 - 0:00 PDT

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A $1 million grant from the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health will fund research at Virginia Tech to identify the cell-level mechanisms that start Duchenne Muscular Dystrophy, a progressive and fatal muscle weakness disease that occurs in approximately one in 3,500 males.

The research project will focus on determining specific mechanisms responsible for Duchenne Muscular Dystrophy early in life so that researchers can develop therapeutic approaches that will blunt or eliminate its onset, said researcher Robert Grange, assistant professor of human nutrition, foods, and exercise in the College of Agriculture and Life Sciences, who will lead a group of scientists from several universities.

Working on the project are Jay Williams, professor of human nutrition, foods, and exercise at Virginia Tech; David Thomas, professor and head, division of structural biology and biophysics, and Dawn Lowe, assistant professor of physical medicine and rehabilitation, both at the University of Minnesota; and Chris Ward, assistant professor of biomedical science at the University of Maryland at Baltimore.

"The goal is to find the mechanisms in the muscle cell by which Duchenne Muscular Dystrophy is initiated," Grange said. Researchers have known that Duchenne Muscular Dystrophy is the result of defects in the gene that codes for the protein dystrophin, but it is still not clear why the muscles weaken and die. This work is to focus on one of the theories about this disease.

This study is looking at possible changes in the outer layer, or membrane of the muscle cell where dystrophin is normally found. When dystrophin is absent, the muscle cell does not work the same and eventually dies. The researchers will look at possible changes in the muscle membrane that may change the way signals are transmitted between the outside and inside of the cell.

These changes in signaling could affect what can be referred to as the engine responsible for muscle contraction, so that contraction is much weaker. One of the signals that may be changed is calcium, which acts as a switch to turn contraction on and off. If the amount of calcium is not carefully controlled in the cell, muscle weakness could occur and the cell could die. The researchers hope that this study will help them better understand if the cell's failure to properly control calcium is what leads to the start of Duchenne Muscular Dystrophy.

Preliminary work by Grange and his associates has been funded by the Muscular Dystrophy Association and the Thomas and Kate Jeffress Memorial Trust.

"Dr. Grange is testing hypotheses that will change our understanding of Duchenne Muscular Dystrophy and, hopefully, lead to new treatments for this fatal muscle disease," said Gerald Jubb, interim head of human nutrition, foods, and exercise at Virginia Tech. "Research on muscle tissue in the College of Agriculture and Life Sciences is enhancing our understanding in many areas of muscle physiology and performance, such as, reducing the risk of microtrauma to muscle tissues through better nutrition and exercise regimes; understanding the role of post-exercise nutrient consumption on muscle protein metabolism and lean tissue gain; and understanding the short- and long-term effects of exercise, inactivity and disease on the functional aspects of neuromuscular system, and skeletal and cardiac muscle. This research impacts all Virginians and helps the College enhance the quality of life for all."

Consistently ranked by the National Science Foundation among the top 10 institutions in agricultural research, Virginia Tech's College of Agriculture and Life Sciences offers students the opportunity to learn from some of the world's leading agricultural scientists. The college's comprehensive curriculum gives students a balanced education that ranges from food and fiber production to economics to human health. The college is a national leader in incorporating technology, biotechnology, computer applications, and other recent scientific advances into its teaching program.

Contact: Mary Ann Johnson
jnayram@vt.edu
540-231-6975
Virginia Tech

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