Electrogenesis of end-plates of mdx mice diaphragm: effect of cell therapy

Natalya Timonina; Violetta Kravtsova; Elena Mikhailova; Anastasia Sokolova; Vyacheslav Mikhailov; Igor Krivoi

doi:10.21638/spbu03.2015.306

Authors

Natalya Timonina Saint Petersburg State University, 7–9, Universitetskaya nab., Saint Petersburg, 199034, Russian Federation
Violetta Kravtsova Saint Petersburg State University, 7–9, Universitetskaya nab., Saint Petersburg, 199034, Russian Federation
Elena Mikhailova Saint Petersburg State University, 7–9, Universitetskaya nab., Saint Petersburg, 199034, Russian Federation
Anastasia Sokolova Institute of Cytology Russian Academy of Science, 4, Tikhoretsky pr., St. Petersburg, 194064, Russian Federation
Vyacheslav Mikhailov Institute of Cytology Russian Academy of Science, 4, Tikhoretsky pr., St. Petersburg, 194064, Russian Federation
Igor Krivoi Saint Petersburg State University, 7–9, Universitetskaya nab., Saint Petersburg, 199034, Russian Federation

DOI:

https://doi.org/10.21638/spbu03.2015.306

Abstract

Disturbances of muscle electrogenesis are observed in a number of muscle pathologies, particularly, they are found in the mdx mice, which are a laboratory model of Duchenne myodystrophy. This myodystrophy develops due to mutations in the gene of the dystrophin protein, which controls the synthesis of this protein in the cytoskeleton. The effectiveness of therapy for such myodystrophy by methods of cellular and genetic engineering has not been studied with regard to the muscle membrane electrogenesis. In this study, two months old mdx mice were irradiated by X-ray at a dose of 3 Gy and injected intravenously by wild type bone marrow cells suspension from long bones of C57Bl/6 mice. Four months after such non-myeloablative bone marrow cells transplantation the recovery of resting membrane potentials as well as parameters of miniature end-plate potentials of mdx mice diaphragm muscle was observed. Our data show the effectiveness of the replacement of the mutant bone marrow with the bone marrow of wild type in myodystrophy caused by the deficiency in dystrophin protein synthesis. Refs 20. Figs 3. Table 1.

Keywords:

mdx mice, dystrophin, end-plate electrogenesis, non-myeloablative bone marrow transplantation

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References

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