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
| Autor: | George Karpati, K.R. Walker, L. Davies, Ros Quinlivan, E. Dunton, K. E. Creed, J.Mc.C. Howell |
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| Rok vydání: | 2013 |
| Předmět: |
Male
medicine.medical_specialty Contraction (grammar) Necrosis Time Factors Phosphorylases Blotting Western Neurotoxins Biology Glycogen phosphorylase chemistry.chemical_compound Internal medicine medicine Animals Regeneration Muscle Strength Muscle Skeletal Genetics (clinical) Elapid Venoms Sheep Muscle fatigue Glycogen Glycogen Phosphorylase medicine.disease Isoenzymes Endocrinology Neurology chemistry Myophosphorylase Pediatrics Perinatology and Child Health Muscle Fatigue Glycogen Storage Disease Type V Fatiguability Neurology (clinical) medicine.symptom Glycogen storage disease type V |
| Zdroj: | Neuromuscular disorders : NMD. 24(2) |
| ISSN: | 1873-2364 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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