Article
Exercise capacity of mice genetically lacking muscle glycogen synthase: in mice, muscle glycogen is not essential for exercise.
Department of Biochemistry and Molecular Biology and Indiana University Center for Diabetes Research, Indiana University School of Medicine, Indianapolis, Indiana 46202-5122, USA.
Journal of Biological Chemistry (impact factor:
4.77).
05/2005;
280(17):17260-5.
DOI:10.1074/jbc.M410448200
pp.17260-5
Source: PubMed
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Citations (0)
- Cited In (4)
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Article: Creatine supplementation spares muscle glycogen during high intensity intermittent exercise in rats.
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ABSTRACT: The effects of creatine (CR) supplementation on glycogen content are still debatable. Thus, due to the current lack of clarity, we investigated the effects of CR supplementation on muscle glycogen content after high intensity intermittent exercise in rats. First, the animals were submitted to a high intensity intermittent maximal swimming exercise protocol to ensure that CR-supplementation was able to delay fatigue (experiment 1). Then, the CR-mediated glycogen sparing effect was examined using a high intensity intermittent sub-maximal exercise test (fixed number of bouts; six bouts of 30-second duration interspersed by two-minute rest interval) (experiment 2). For both experiments, male Wistar rats were given either CR supplementation or placebo (Pl) for 5 days. As expected, CR-supplemented animals were able to exercise for a significant higher number of bouts than Pl. Experiment 2 revealed a higher gastrocnemius glycogen content for the CR vs. the Pl group (33.59%). Additionally, CR animals presented lower blood lactate concentrations throughout the intermittent exercise bouts compared to Pl. No difference was found between groups in soleus glycogen content. The major finding of this study is that CR supplementation was able to spare muscle glycogen during a high intensity intermittent exercise in rats.Journal of the International Society of Sports Nutrition 01/2010; 7(1):6. · 1.64 Impact Factor -
Article: PTG depletion removes Lafora bodies and rescues the fatal epilepsy of Lafora disease.
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ABSTRACT: Lafora disease is the most common teenage-onset neurodegenerative disease, the main teenage-onset form of progressive myoclonus epilepsy (PME), and one of the severest epilepsies. Pathologically, a starch-like compound, polyglucosan, accumulates in neuronal cell bodies and overtakes neuronal small processes, mainly dendrites. Polyglucosan formation is catalyzed by glycogen synthase, which is activated through dephosphorylation by glycogen-associated protein phosphatase-1 (PP1). Here we remove PTG, one of the proteins that target PP1 to glycogen, from mice with Lafora disease. This results in near-complete disappearance of polyglucosans and in resolution of neurodegeneration and myoclonic epilepsy. This work discloses an entryway to treating this fatal epilepsy and potentially other glycogen storage diseases.PLoS Genetics 04/2011; 7(4):e1002037. · 8.69 Impact Factor -
Article: Muscle glycogen storage disease 0 presenting recurrent syncope with weakness and myalgia.
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ABSTRACT: Muscle glycogen storage disease 0 (GSD0) is caused by glycogen depletion in skeletal and cardiac muscles due to deficiency of glycogen synthase 1 (GYS1), which is encoded by the GYS1 gene. Only two families with this disease have been identified. We report a new muscle GSD0 patient, a Japanese girl, who had been suffering from recurrent attacks of exertional syncope accompanied by muscle weakness and pain since age 5 years until she died of cardiac arrest at age 12. Muscle biopsy at age 11 years showed glycogen depletion in all muscle fibers. Her loss of consciousness was gradual and lasted for hours, suggesting that the syncope may not be simply caused by cardiac event but probably also contributed by metabolic distress.Neuromuscular Disorders 09/2011; 22(2):162-5. · 2.80 Impact Factor
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Keywords
exercise endurance
exhaustion
Fasting
glucose storage polymer glycogen
glycogen
glycogen synthase
liver glycogen
MGSKO mice
MGSKO-null mice
mice muscle glycogen
mouse model
muscle glycogen
muscle isoform
normal
quadriceps muscles
skeletal muscle
skeletal muscle contraction
strenuous exercise
wild type animals
wild type littermates