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 journalArticle

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

Fingerprint

Glucosephosphate Dehydrogenase Deficiency
Glucosephosphate Dehydrogenase
Skeletal Muscle Fibers
Myoblasts
Muscles
Muscular Diseases
Cell Culture Techniques
Transplantation
Staining and Labeling

Keywords

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

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Muscle glucose-6-phosphate dehydrogenase deficiency : Restoration of enzymatic activity in hybrid myotubes. / Meola, G.; Tremblay, J. P.; Sansone, V.; Rotondo, G.; Radice, S.; Bresolin, N.; Huard, J.; Scarlato, G.

In: Muscle and Nerve, Vol. 16, No. 6, 1993, p. 594-600.

Research output: Contribution to journalArticle

Meola, G. ; Tremblay, J. P. ; Sansone, V. ; Rotondo, G. ; Radice, S. ; Bresolin, N. ; Huard, J. ; Scarlato, G. / Muscle glucose-6-phosphate dehydrogenase deficiency : Restoration of enzymatic activity in hybrid myotubes. In: Muscle and Nerve. 1993 ; Vol. 16, No. 6. pp. 594-600.
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