AI Article Synopsis
- McArdle disease is due to a lack of myophosphorylase, and effective treatments are currently unavailable.
- Injecting notexin into affected sheep muscles led to muscle damage followed by regeneration, increasing muscle strength and reducing fatigue.
- The restoration of non-muscle phosphorylase isoforms suggests that therapies aimed at enhancing these isoforms could be promising for treating McArdle disease.
Article 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.
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| http://dx.doi.org/10.1016/j.nmd.2013.10.003 | DOI Listing |
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