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

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 expres...

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Veröffentlicht in:	Neuromuscular disorders : NMD 2014-02, Vol.24 (2), p.167-177
Hauptverfasser:	Howell, J.McC, Walker, K.R, Creed, K.E, Dunton, E, Davies, L, Quinlivan, R, Karpati, G
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Blotting, Western Elapid Venoms - toxicity Glycogen - metabolism Glycogen phosphorylase Glycogen Phosphorylase - metabolism Glycogen Storage Disease Type V - physiopathology Isoenzymes Male McArdle disease Muscle Fatigue - physiology Muscle Strength - physiology Muscle, Skeletal - drug effects Muscle, Skeletal - physiopathology Myophosphorylase Necrosis - chemically induced Necrosis - physiopathology Neurology Neurotoxins - toxicity Notexin Ovine model of McArdle disease Phosphorylase isoforms Phosphorylases - metabolism Regeneration Sheep Time Factors
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