Muscle glucose-6-phosphate dehydrogenase deficiency: Restoration of enzymatic activity in hybrid myotubes

G. Meola, J. P. Tremblay, V. Sansone, G. Rotondo, S. Radice, N. Bresolin, J. Huard, G. Scarlato

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)594-600
Number of pages7
JournalMuscle and Nerve
Volume16
Issue number6
DOIs
Publication statusPublished - 1993

Keywords

  • fluorescent latex microspheres
  • glucose-6-phosphate dehydrogenase
  • hybrid myotubes
  • muscle cell culture
  • myoblast transplantation

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

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