March 13, 2009

Lecturer tracks path of muscular dystrophy research

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Discovery Lecturer Kevin Campbell, Ph.D., right, talks with Billy Hudson, Ph.D., following his talk last week. (photo by Susan Urmy)

Lecturer tracks path of muscular dystrophy research

At last week's Discovery Lecture, Kevin Campbell, Ph.D., detailed his work on the genetic defects that cause muscular dystrophy — a group of genetic diseases that primarily affects skeletal muscle and leads to progressive muscle weakness.

Campbell, who is the director of the Wellstone Muscular Dystrophy Research Center and chair of the Department of Molecular Physiology and Biophysics at the University of Iowa, and his colleagues isolated and characterized a major complex of proteins, called the dystrophin-glycoprotein complex (DGC). Many of the proteins in this complex are mutated in various forms of muscular dystrophy.

“That discovery was a breakthrough at the basic science level that represented the first step in clarifying the pathogenesis of muscular dystrophy,” said Billy Hudson, Ph.D., director of the Center for Matrix Biology, in his introduction of Campbell.

Campbell — a Howard Hughes Medical Institute investigator and a member of the Institute of Medicine and the National Academy of Sciences — described how mutations and protein processing changes in the various complex components cause cellular disruptions and lead to muscle damage.

Specifically, he demonstrated how defects in DGC function disrupt the link between the extracellular matrix and the internal “skeleton” of muscle cells. This broken link makes the muscle membrane more susceptible to injury and leads to progressive muscle weakness.

Citing his basic science findings as well as his investigations of human cases of muscular dystrophy, Campbell noted that improved understanding of the molecular causes of the different forms of muscular dystrophy is providing new therapeutic strategies for treating these diseases.

Campbell's talk was also the Ernest W. Goodpasture Lecture in Investigative Pathology, which honors the pioneering Vanderbilt pathologist best known for his work on virus culture techniques. The lecture was sponsored by the Department of Pathology and the Center for Matrix Biology.