Altered ribosomal protein synthesis in congenital non-progressive myopathy

Ribosomes isolated from fibroblasts, muscle tissues, and blood cells of a patient with congenital non-progressive myopathy were used for in vitro measurement of protein synthesis in a heterologous poly(U)-directed polyphenylalanine synthesis system. The activity of ribosomes obtained from the patient was 35% lower than that in normal controls.