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Stimulatory effects of drugs for protein synthesis on muscle cell cultures in duchenne dystrophy
Author(s) -
Ionasescu Victor,
Stern Lawrence Z.,
Ionasescu Rebeca,
Rubenstein Peter
Publication year - 1979
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410050202
Subject(s) - duchenne muscular dystrophy , protein biosynthesis , myosin , cell , muscular dystrophy , cell culture , endocrinology , leucine , medicine , myocyte , biochemistry , chemistry , microbiology and biotechnology , biology , amino acid , genetics
The influence of the membrane‐stablizing agents diphenylhydantoin (DPH) and orgotein (a drug with superoxide dismutase activity) on protein synthesis was studied in cultured human muscle cells obtained from 7 patients with Duchenne muscular dystrophy (DMD) and 14 controls. The cultures were obtained by dissociation and subsequent plating of cells from biopsied quadriceps muscle. These cultures were then labeled with tritiated leucine in the presence and absence of the membrane‐stabilizing drugs. Total protein synthesis (cpm/mg noncollagen protein) in first‐passage muscle cell cultures from DMD patients showed a 35% decrease, but myosin synthesis revealed normal values. DPH and orgotein increased total protein synthesis by 35 and 50%, respectively, in dystrophic cultured cells, but only by 7 and 8%, respectively, in control cultured cells.