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Archive - Aug 2, 2013

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Trace Substance in Caramelized Sugar Improves Muscle Regeneration in Fly and Mousel Models of Muscular Dystrophy

A trace substance in caramelized sugar, when purified and given in appropriate doses, improves muscle regeneration in a mouse model of Duchenne muscular dystrophy. The findings were published online on August 1, 2013 in an open-access article in the journal Skeletal Muscle. Dr. Morayma Reyes, professor of pathology and laboratory medicine, and Dr. Hannele Ruohola-Baker, professor of biochemistry and associate director of the Institute for Stem Cell and Regenerative Medicine, headed the University of Washington (UW) team that made the discovery. The first authors of the paper were Dr. Nicholas Ieronimakis, UW Department of Pathology; and Dr. Mario Pantoja, UW Department of Biochemistry. The authors explained that the mice in their study, like boys with the gender-linked inherited disorder muscular dystrophy, are missing the gene that produces dystrophin, a muscle-repair protein. Neither the mice nor the affected boys can replace enough of their routinely lost muscle cells. In people, muscle weakness begins when the boys are toddlers, and progresses until, as teens, they can no longer walk unaided. During early adulthood, their heart and respiratory muscles weaken. Even with ventilators to assist breathing, death usually ensues before age 30. No cure or satisfactory treatment is available. Prednisone drugs relieve some symptoms, but at the cost of severe side effects. The disabling, then lethal, nature of the rare disease in young men presses scientists to search for better therapeutic agents. Dr. Reyes and Dr. Ruohola-Baker are seeking ways to suppress the disorder's characteristic functional and structural muscle defects. Dr. Ruohola-Baker's lab originally identified the sphingosine 1-phosphate (S1P) pathway as a critical player in ameliorating muscular dystrophy in flies.