Biochemical changes in hereditary progressive muscular dystrophies. Defect of protein synthesis in fibroblasts, muscle tissues and blood cells.
80S ribosomes and ribosomal subunits were isolated from fibroblasts, muscle tissues and blood cells of patients with different muscular dystrophies (MD) as well as of controls and were used for in vitro measurement of ribosomal protein synthesis (RPS) in a poly(U)-directed polyphenylalanine synthesis system. The activity of ribosomes from the patients showed a disease-dependent decrease compared to normal controls. Examination of hybrid 80S ribosomes consisting of 40S and 60S subunits of patients and the corresponding control cells revealed that the loss of RPS activity was related to one or both of the ribosomal subunits depending on the type of MD.
Pöche
H
H
Neurologische Klinik, Universitätsklinikum Steglitz, Freie Universität Berlin.
Kattner
E
E
Marx
P
P
D001773:Blood Cells / Q000378:metabolism*; D005347:Fibroblasts / Q000378:metabolism; D006801:Humans; D008297:Male; D009132:Muscles / Q000378:metabolism*; D009136:Muscular Dystrophies / Q000235:genetics* / Q000378:metabolism; D010452:Peptide Biosynthesis; D010455:Peptides; D012269:Ribosomal Proteins / Q000096:biosynthesis*; D012270:Ribosomes / Q000378:metabolism*
D010455:Peptides; D012269:Ribosomal Proteins; C012001:polyphenylalanine