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 | |
| Publication status | Published - 1993 |
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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 journal › Article
}
TY - JOUR
T1 - Muscle glucose-6-phosphate dehydrogenase deficiency
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
UR - http://www.scopus.com/inward/record.url?scp=0027231079&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0027231079&partnerID=8YFLogxK
U2 - 10.1002/mus.880160604
DO - 10.1002/mus.880160604
M3 - Article
VL - 16
SP - 594
EP - 600
JO - Muscle and Nerve
JF - Muscle and Nerve
SN - 0148-639X
IS - 6
ER -