Muscle cells of untreated mice with muscular dystrophy (left) show little utrophin in cell walls while those of mice treated with biglycan (right) have accumulated the protein (Credit: Fallon Lab/Brown University)
Duchenne Muscular Dystrophy is the most common and severe childhood form of muscular dystrophy (MD), affecting one in 3,500 boys. The disease progressively weakens muscles cells and tissues until muscle degradation is so severe that the patient dies, most often in their late teens or twenties. Scientists at Brown University in Providence, Rhode Island and the University of Pennsylvania, hope their research into the human protein, biglycan, will ultimately improve the condition of muscular dystrophy sufferers. Their studies have shown that biglycan significantly slows muscle damage and improves function in mice with the Duchenne genetic mutation. Human clinical trials will be the next step.