Muscle Deterioration Studied
Studies of a protein linked to the most serious form of muscular dystrophy suggest the disease may kill muscle by weakening muscle cell walls, an idea that may help lead to treatments.
The protein is absent from muscles of people with Duchenne dystrophy, and the studies suggest that may result in abnormally weak cell walls.
In the journal Nature, Louis Kunkel of the Howard Hughes Medical Institute at Children’s Hospital in Boston, and colleagues, said they are trying to determine the function of the protein, called dystrophin, so they can tell just why its absence is so destructive in Duchenne. That knowledge is expected to guide development of treatments.
The studies do not directly demonstrate dystrophin’s function, but hint at it by showing where the protein appears within the muscle cell. The results support the cell-wall hypothesis, proposed before dystrophin was discovered last year.
Similar conclusions were published last month by Canadian researchers, including Elizabeth Zubrzycka-Gaarn and Ronald Worton of the Hospital for Sick Children in Toronto and the University of Toronto.
