51. Muscle glucose-6-phosphate dehydrogenase deficiency: restoration of enzymatic activity in hybrid myotubes
- Author
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Valeria A. Sansone, Johnny Huard, Nereo Bresolin, S. Radice, Jacques P. Tremblay, Giovanni Meola, Guglielmo Scarlato, and G. Rotondo
- Subjects
Adult ,congenital, hereditary, and neonatal diseases and abnormalities ,Physiology ,Dehydrogenase ,Biology ,Glucosephosphate Dehydrogenase ,Hybrid Cells ,Muscle Development ,Microtubules ,Cellular and Molecular Neuroscience ,chemistry.chemical_compound ,Mice ,hemic and lymphatic diseases ,Physiology (medical) ,parasitic diseases ,Glucose-6-phosphate dehydrogenase ,Myocyte ,Animals ,Humans ,Cells, Cultured ,chemistry.chemical_classification ,Myogenesis ,Muscles ,nutritional and metabolic diseases ,musculoskeletal system ,Molecular biology ,Staining ,Transplantation ,Enzyme Activation ,Enzyme ,Glucosephosphate Dehydrogenase Deficiency ,Biochemistry ,chemistry ,Cell culture ,Neurology (clinical) - Abstract
A high level of glucose-6-phosphate dehydrogenase (G6PD) activity was observed in myoblasts and myotubes from normal human and mouse cell cultures. However, only a residual amount of activity was observed in myoblasts and myotubes obtained from G6PD-deficient patients (G6PD Mediterranean). Hybrids were formed by the fusion of normal (from human and mouse) and G6PD-deficient myoblasts (from the patients). These hybrids contained a high level of G6PD activity. Hoechst staining permitted to confirm that the enzymatic activity was not restrained to a domain near the competent nuclei. These results suggest that myoblast transplantation could be used to restore normal enzymatic activity in metabolic myopathies.
- Published
- 1993