MITOCHONDRIAL MYOPATHY: A NEW THERAPEUTIC APPROACH

Authors

  • B.A. HAGIU 1. University “Al. I. Cuza”-Iaşi
  • O.C. MUNGIU University of Medicine and Pharmacy “Grigore. T. Popa”-Iaşi

Abstract

Restoration of deoxyribonucleic acid in mitochondrial myopathies may occur after a mechanical or chemical injury of striated muscle or by endurance training. Therapies with enzymes, gene therapies, or treatments with substances that stimulate mitochondrial biogenesis are used at the moment. Genesis of mitochondria may also come from myonuclei by releasing the nuclear respiratory factor-1/2 during muscle contractions. Multiplying of myonuclei depends on muscle satellite cell activation. Since the electromyostimulation increase the number of circulating stem cells that may participate in the genesis of new muscle fibers (adding to the deposit of specific stem cells of the muscle), and intermittent hypoxia stimulates the proliferation of muscle satellite cells, we propose to combine the two processes for the treatment of mitochondrial myopathies. Respective combined therapy may be useful for restoring damaged mitochondria by drug side effects.

Author Biography

  • O.C. MUNGIU, University of Medicine and Pharmacy “Grigore. T. Popa”-Iaşi

    Faculty of Medicine
    Department of Morpho-functional Sciences

References

1. Clark KM et al. Reversal of a mitochondrial DNA defect in human skeletal muscle. Nat. Genet. 1997; 16: 222–224.
2. Shoubridge EA, Johns T, Karpati G. Complete restoration of a wild-type mtDNA genotype in regen-erating muscle fibres in a patient with a tRNA point mutation and mitochondrial encephalomyopa-thy. Hum. Mol. Genet. 1997; 6: 2239–2242.
3. Taivassalo T, Fu K, Johns T, Arnold D, Karpati G, Soubridge EA. Gene shifting:a novel therapy for mitochondrial myopathy. Hum. Mol. Genet. 1999; 8: 1047–1052.
4. Murphy JLet al. Resistance training in patients with single, large-scale deletions of mitochondrial DNA. Brain 2008; 131: 2832–2840.
5. Poole OV, Hanna MG, Pitceathly RD. Mitochondrial disorders: disease mechanisms and therapeutic approaches. Discov Med. 2015; 20(111): 325-331.
6. Jornayvaz FR, Shulman GI. Regulation of mitochondrial biogenesis. Essays in biochemistry 2010; 47: 10.1042/bse0470069.
7. Bergeron R et al. Chronic activation of AMP kinase results in NRF-1 activation and mitochondrial biogenesis. Am J Physiol Endocrinol Metab. 2001; 281(6): E1340-E1346.
8. Claude Bouchard (ed). Molecular and cellular regulation of adaptation to exercise. Progress in molecular biology and translational science - Vol 135. Academic Press (Elsevier) USA, UK 2015.
9. Yin H, Price F, Rudnicki MA. Satellite Cells and the Muscle Stem Cell Niche. Physiological Rev 2013; 93(1): 23-67.
10. Anderson J, Pilipowicz O. Activation of muscle satellite cells in single-fiber cultures. Nitric Ox-ide 2002; 7(1): 36-41.
11. Carlson ME, Suetta C, Conboy MJ, et al. Molecular aging and rejuvenation of human muscle stem cells. EMBO Molec Med 2009; 1(8-9): 381-391.
12. Booth FW. Effects of endurance exercise on cytochrome c turnover în skeletal muscle. Annals N. Y. Acad. Sci 1977; 301: 431-439.
13. Cabric M, Appel HJ, Resic A. Effects of Electrical Stimulation of Different Frequencies on the Myo-nuclei and Fiber Size in Human Muscle. Intern J Sports Med 1987; 8(5):323-326.
14. Viscor G et al Combined intermittent hypoxia and surface muscle electrostimulation as a method to increase peripheral blood progenitor cell concentration. J Transl Med 2009; 7: 91-95.
15. Motohashi N, Asakura A. Muscle satellite cell heterogeneity and self-renewal. Front Cell Dev Bi-ol 2014; 2:1.
16. Spendiff S et al. Mitochondrial DNA deletions in muscle satellite cells: implications for therapies. Hum Mol Genet 2013; 22: 4739–4747.
17. Deley G, Babault N. Could Low-Frequency Electromyostimulation Training be an Effective Alternative to Endurance Training? An Overview in One Adult. J Sports Sci Med 2014; 13(2): 444-450.
18. Belenky P. et al. "NAD+ Metabolism in Health and Disease". Trends in Biochemic Sci 2007; 32: 12–19.
19. Cantó C, Keir J. Menzies KJ, Auwerx J. NAD+ Metabolism and the Control of Energy Homeostasis: A Balancing Act between Mitochondria and the Nucleus. Cell Metabolism 2015; 22(1): 31–53.
20. Neustadt J, Pieczenik SR. Medication-induced mitochondrial damage and disease. Mol. Nutr. Food Res. 2008; 52: 780-788.

Additional Files

Published

2016-06-30

Issue

Vol. 120 No. 2 (2016): The Medical-Surgical Journal

Section

BASIC SCIENCES

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