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

J.Mc.C. Howell; K.R. Walker; K.E. Creed; E. Dunton; L. Davies; R. Quinlivan; G. Karpati

doi:10.1016/j.nmd.2013.10.003

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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

Journal article

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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

J.Mc.C. Howell, K.R. Walker, K.E. Creed, E. Dunton, L. Davies, R. Quinlivan and G. Karpati

Neuromuscular Disorders, Vol.24(2), pp.167-177

2013

DOI: https://doi.org/10.1016/j.nmd.2013.10.003

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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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Title: 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
Authors/Creators: J.Mc.C. Howell (Author/Creator) - The University of Western Australia
K.R. Walker (Author/Creator) - The University of Western Australia
K.E. Creed (Author/Creator) - Murdoch University
E. Dunton (Author/Creator) - Murdoch University
L. Davies (Author/Creator) - Murdoch University
R. Quinlivan (Author/Creator) - National Hospital for Neurology and Neurosurgery
G. Karpati (Author/Creator) - Montreal Neurological Institute and Hospital
Publication Details: Neuromuscular Disorders, Vol.24(2), pp.167-177
Publisher: Elsevier BV
Identifiers: 991005540313307891
Copyright: © 2013 Elsevier B.V.
Murdoch Affiliation: School of Psychology and Exercise Science
Language: English
Resource Type: Journal article

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Collaboration types: Domestic collaboration; International collaboration
Citation topics: 1 Clinical & Life Sciences; 1.271 Lysosomal Storage Disorders; 1.271.2265 Glycogen Storage Diseases
Web Of Science research areas: Clinical Neurology; Neurosciences
ESI research areas: Neuroscience & Behavior