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Phosphorylase expression in McArdle’s sheep following notexin injections and in Recumbent Animals

Howell, J.Mc.C., Davies, L., Fletcher, S., Honeyman, K. and Wilton, S. (2006) Phosphorylase expression in McArdle’s sheep following notexin injections and in Recumbent Animals. Brain Pathology, 13 . S107.

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Background. McArdle’s disease is an inherited myopathy caused by a deficiency of myophosphorylase. Sheep with the disease are being used to study gene therapy regimes and upregulation of phosphorylase. Methods. One hundred microliters of a 5 g/ml solution of notexin was injected into the semitendinosus muscles of 20 affected sheep, 16 aged from 1 to 6 days and 4 from 2 to 3 years. Biopsies were taken 10, 30, and 60 days after the injections. Muscle samples were also taken from 2 mature affected sheep were recumbent for 11 and 16 days. Frozen sections from the muscle samples were stained with H&E and for phosphorylase activity. Results. Myophosphorylase-positive fibers were seen in areas of muscle damage and regeneration in all the 10 day biopsies from the 16 lambs and 4 mature sheep. Similarly stained fibers were seen in biopsies taken after 30 and 60 days. However the number of positive fibers diminished with time. Myophosphorylase-positive fibers were also seen in muscles of the recumbent, uninjected, mature sheep. The number of positive fibers varied from muscle to muscle but were most frequent in the extra ocular and extensor carpi radialis, whereas none were seen in the masseter muscles. Conclusions. Regeneration occurs in muscles of sheep with McArdle’s disease and it is accompanied by upregulation of phosphorylase.

Item Type: Journal Article
Murdoch Affiliation(s): School of Veterinary and Biomedical Sciences
Publisher: Wiley
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