Premium
Muscle glucose‐6‐phosphate dehydrogenase deficiency: Restoration of enzymatic activity in hybrid myotubes
Author(s) -
Meola Giovanni,
Tremblay Jacques P.,
Sansone Valeria,
Rotondo, Giuseppe,
Radice Stefania,
Bresolin Nereo,
Huard Johnny,
Scarlato Guglielmo
Publication year - 1993
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.880160604
Subject(s) - myogenesis , glucose 6 phosphate dehydrogenase , myocyte , dehydrogenase , enzyme , enzyme assay , biology , biochemistry , chemistry , microbiology and biotechnology , medicine , endocrinology
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.