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

doi:10.1002/mus.880160604

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 journal › Article › peer-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 language	English
Pages (from-to)	594-600
Number of pages	7
Journal	Muscle and Nerve
Volume	16
Issue number	6
DOIs	https://doi.org/10.1002/mus.880160604
Publication status	Published - 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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10.1002/mus.880160604

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title = "Muscle glucose-6-phosphate dehydrogenase deficiency: Restoration of enzymatic activity in hybrid myotubes",

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.",

keywords = "fluorescent latex microspheres, glucose-6-phosphate dehydrogenase, hybrid myotubes, muscle cell culture, myoblast transplantation",

author = "G. Meola and Tremblay, {J. P.} and V. Sansone and G. Rotondo and S. Radice and N. Bresolin and J. Huard and G. Scarlato",

year = "1993",

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language = "English",

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T2 - Restoration of enzymatic activity in hybrid myotubes

AU - Meola, G.

AU - Tremblay, J. P.

AU - Sansone, V.

AU - Rotondo, G.

AU - Radice, S.

AU - Bresolin, N.

AU - Huard, J.

AU - Scarlato, G.

PY - 1993

Y1 - 1993

N2 - 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.

AB - 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.

KW - fluorescent latex microspheres

KW - glucose-6-phosphate dehydrogenase

KW - hybrid myotubes

KW - muscle cell culture

KW - myoblast transplantation

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