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Neuromuscul Disord. 2014 Feb;24(2):167-77. doi: 10.1016/j.nmd.2013.10.003. Epub 2013 Oct 26.

Phosphorylase re-expression, increase in the force of contraction and decreased fatigue following notexin-induced muscle damage and regeneration in the ovine model of McArdle disease.

Author information

1
School of Veterinary and Life Sciences, Murdoch University, Perth 6150, Western Australia, Australia; Australian Neuro-Muscular Research Institute,CNND, University of Western Australia, Perth 6150, Western Australia, Australia. Electronic address: J.Howell@murdoch.edu.au.
2
Centre for Medical Research, University of Western Australia, Perth 6150, Western Australia, Australia.
3
School of Veterinary and Life Sciences, Murdoch University, Perth 6150, Western Australia, Australia.
4
Centre for Neuromuscular Diseases, National Hospital for Neurology and Neurosurgery, London, United Kingdom.
5
Montreal Neurological Institute, McGill University, Montreal, Quebec, Canada.

Abstract

McArdle disease is caused by a deficiency of myophosphorylase and currently a satisfactory treatment is not available. The injection of notexin into, or the layering of notexin onto, the muscles of affected sheep resulted in necrosis followed by regeneration of muscle fibres with the expression of both non-muscle isoforms of phosphorylase within the fibres and a reduction of the amount of glycogen in the muscle with an increase in the strength of contraction and a decrease in fatiguability in the muscle fibres. The sustained re-expression of both the brain and liver isoforms of phosphorylase within the muscle fibres provides further emphasis that strategies to enhance the re-expression of these isoforms should be investigated as a possible treatment for McArdle disease.

KEYWORDS:

Glycogen phosphorylase; McArdle disease; Myophosphorylase; Notexin; Ovine model of McArdle disease; Phosphorylase isoforms.

PMID:
24309536
DOI:
10.1016/j.nmd.2013.10.003
[Indexed for MEDLINE]

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