Protein degradation in cultured skeletal muscle from Duchenne muscular dystrophy patients.

The loss of contractile protein in Duchenne muscular dystrophy could result from low rates of synthesis, abnormally high rates of protein degradation, or a combination of both. We measured overall protein degradation rates in cultured human muscle cells obtained at biopsy from patients with Duchenne dystrophy or various muscle diseases and normal subjects. Measurements were performed on confluent cultures exhibiting no growth and containing a mixed cell population of myoblasts, fibroblasts, and multinucleated myotubes. Using a new double-isotope labeling protocol, we found protein degradation rates in all three groups to be similar (KD = 0.0171-0.0176 hr-1), suggesting no detectable abnormality of overall protein degradation in cells derived from Duchenne dystrophy patients.