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C L Bodinham
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L Smith
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E L Thomas Nutrition, Metabolic and Molecular Imaging Group, Department of Food and Nutritional Sciences, Metabolism and Diabetes Research Group, Faculty of Health and Medical Sciences, University of Surrey, Leggett Building, Guildford, Surrey GU2 7WG, UK

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J D Bell Nutrition, Metabolic and Molecular Imaging Group, Department of Food and Nutritional Sciences, Metabolism and Diabetes Research Group, Faculty of Health and Medical Sciences, University of Surrey, Leggett Building, Guildford, Surrey GU2 7WG, UK

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J R Swann Nutrition, Metabolic and Molecular Imaging Group, Department of Food and Nutritional Sciences, Metabolism and Diabetes Research Group, Faculty of Health and Medical Sciences, University of Surrey, Leggett Building, Guildford, Surrey GU2 7WG, UK

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A Costabile Nutrition, Metabolic and Molecular Imaging Group, Department of Food and Nutritional Sciences, Metabolism and Diabetes Research Group, Faculty of Health and Medical Sciences, University of Surrey, Leggett Building, Guildford, Surrey GU2 7WG, UK

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D Russell-Jones
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A M Umpleby
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M D Robertson
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the HAM-RS2 ( P =0.036). The A-V sampling across the forearm muscle also showed a trend for higher glucose uptake with HAM-RS2 compared with the placebo ( P =0.077; Fig. 1 B). Figure 1 Postprandial glucose concentrations (A), glucose flux into the

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Leanne Hodson Oxford Centre for Diabetes, Endocrinology and Metabolism, Radcliffe Department of Medicine, University of Oxford and National Institute for Health Research (NIHR) Oxford Biomedical Research Centre (BRC), Oxford University Hospital Trusts, Oxford, UK

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Fredrik Karpe Oxford Centre for Diabetes, Endocrinology and Metabolism, Radcliffe Department of Medicine, University of Oxford and National Institute for Health Research (NIHR) Oxford Biomedical Research Centre (BRC), Oxford University Hospital Trusts, Oxford, UK

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hyperinsulinaemia are often said to have ‘insulin resistance’ ( 4 , 5 ). Here we discuss the direct and indirect effects of insulin on intrahepatic processes (e.g. fatty acid synthesis) and systemic processes (e.g. the regulation of fatty acid flux from adipose

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Nicolai Preisler Copenhagen Neuromuscular Center, Department of Neurology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark

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Pascal Laforêt Centre de Référence de Pathologie Neuromusculaire Paris-Est, Institut de Myologie, GH Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France

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Karen Lindhardt Madsen Copenhagen Neuromuscular Center, Department of Neurology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark

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Edith Husu Copenhagen Neuromuscular Center, Department of Neurology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark

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Christoffer Rasmus Vissing Copenhagen Neuromuscular Center, Department of Neurology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark

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Gitte Hedermann Copenhagen Neuromuscular Center, Department of Neurology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark

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Henrik Galbo Department of Inflammation Research, Rigshospitalet, Copenhagen, Denmark

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Christopher Lindberg Department of Neurology, Sahlgrenska University Hospital, Gothenburg, Sweden

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John Vissing Copenhagen Neuromuscular Center, Department of Neurology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark

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Objective

Pompe disease (glycogenosis type II) is caused by lysosomal alpha-glucosidase deficiency, which leads to a block in intra-lysosomal glycogen breakdown. In spite of enzyme replacement therapy, Pompe disease continues to be a progressive metabolic myopathy. Considering the health benefits of exercise, it is important in Pompe disease to acquire more information about muscle substrate use during exercise.

Methods

Seven adults with Pompe disease were matched to a healthy control group (1:1). We determined (1) peak oxidative capacity (VO2peak) and (2) carbohydrate and fatty acid metabolism during submaximal exercise (33 W) for 1 h, using cycle-ergometer exercise, indirect calorimetry and stable isotopes.

Results

In the patients, VO2peak was less than half of average control values; mean difference −1659 mL/min (CI: −2450 to −867, P = 0.001). However, the respiratory exchange ratio increased to >1.0 and lactate levels rose 5-fold in the patients, indicating significant glycolytic flux. In line with this, during submaximal exercise, the rates of oxidation (ROX) of carbohydrates and palmitate were similar between patients and controls (mean difference 0.226 g/min (CI: 0.611 to −0.078, P = 0.318) and mean difference 0.016 µmol/kg/min (CI: 1.287 to −1.255, P = 0.710), respectively).

Conclusion

Reflecting muscle weakness and wasting, Pompe disease is associated with markedly reduced maximal exercise capacity. However, glycogenolysis is not impaired in exercise. Unlike in other metabolic myopathies, skeletal muscle substrate use during exercise is normal in Pompe disease rendering exercise less complicated for e.g. medical or recreational purposes.

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Ashley N Reeb Department of Otolaryngology, Head and Neck Surgery, Saint Louis University School of Medicine, Saint Louis, Missouri, USA

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Andrea Ziegler Department of Otolaryngology, Head and Neck Surgery, Saint Louis University School of Medicine, Saint Louis, Missouri, USA

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Reigh-Yi Lin Department of Otolaryngology, Head and Neck Surgery, Saint Louis University School of Medicine, Saint Louis, Missouri, USA

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-CO2 cell lines. Stable clones of WRO, FTC-238, and TT2609-CO2 cells expressing luciferase were serially diluted. Luciferin substrate was added to each well 10 min before imaging and the plate was imaged to obtain total flux (p/s) per cell line using

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Jiwen Yang Department of Nuclear Medicine, Yijishan Hospital of Wannan Medical College, Wuhu City, Anhui Province, China

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Yayin Huang Department of Clinical Laboratory, The Second People’s Hospital of Wuhu, Wuhu City, Anhui Province, China

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Bohan Dong Department of Biochemistry and Molecular Biology, Wannan Medical College, Wuhu City, Anhui Province, China

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Yunhai Dai Department of Nuclear Medicine, Yijishan Hospital of Wannan Medical College, Wuhu City, Anhui Province, China

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stained and counted under a microscope. Aerobic glycolysis detection The real-time extracellular acidification rate (ECAR) was detected through an XF96 metabolic flux analyzer (Seahorse Biosciences, Billerica, MA, USA) ( 27 ). TPC-1 and BHT-101

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M Feller R Fernandez M Fernandez Oliveira F Ferrau F Ferraù H Ferrero L Figueroa S Filetti C Fiori S Fischli J Flannick A Fletcher E Fliers G Flux C Follin S Forbes P A Foster L Fozzatti M Fragoso K Frank

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Espen Nordheim Department of Transplantation Medicine, Section of Nephrology, Oslo University Hospital, Rikshospitalet, Oslo, Norway
Faculty of Medicine, University of Oslo, Oslo, Norway

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Trond Geir Jenssen Department of Transplantation Medicine, Section of Nephrology, Oslo University Hospital, Rikshospitalet, Oslo, Norway
Faculty of Medicine, University of Oslo, Oslo, Norway
Metabolic and Renal Research Group, Faculty of Health Sciences, UiT- The Arctic University of Norway, Tromsø

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back-transportation of sodium and glucose from the renal tubular system ( 6 ). Sodium-glucose-co-transporter-2 (SGLT2) contributes to 90% of this transportation reducing distal tubular flux of glucose and sodium. Due to reduced sodium flux in the loop

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Mengxue Yang Department of Endocrinology, The Fifth People’s Hospital of Shanghai, Fudan University, Shanghai, China
Department of Endocrinology, Zunyi Medical University, Zunyi, China

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Bowen Sun Department of Endocrinology, Zunyi Medical University, Zunyi, China

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Jianhui Li Department of Endocrinology, Ningbo No. 2 Hospital, Ningbo, China

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Bo Yang Department of Endocrinology, Zunyi Medical University, Zunyi, China

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Jie Xu School of Public Health, Zunyi Medical University, Zunyi, China

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Xue Zhou Department of Endocrinology, Zunyi Medical University, Zunyi, China

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Jie Yu School of Public Health, Zunyi Medical University, Zunyi, China

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Xuan Zhang Department of Endocrinology, Zunyi Medical University, Zunyi, China

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Qun Zhang Department of Endocrinology, Ningbo No. 2 Hospital, Ningbo, China

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Shan Zhou Department of Endocrinology, Ningbo No. 2 Hospital, Ningbo, China

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Xiaohua Sun Department of Endocrinology, Ningbo No. 2 Hospital, Ningbo, China

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microbial diversity. Statistical analysis The original high-flux sequencing data were initially screened according to the sequence quality. The sequences were identified and then assigned to corresponding samples according to the primer and barcode

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Aasem Saif Internal Medicine Department, Cairo University, Cairo, Egypt

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Shrook Mousa Internal Medicine Department, Cairo University, Cairo, Egypt

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Maha Assem Internal Medicine Department, Cairo University, Cairo, Egypt

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Nashwa Tharwat National Nutrition Institute, Cairo, Egypt

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Alaa Abdelhamid Internal Medicine Department, Cairo University, Cairo, Egypt
Vascular Laboratory, Cairo University, Cairo, Egypt

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same skilled sonographer. Endothelial function has been assessed by measuring the percent of change in blood flow following heat-mediated vasodilation using laser Doppler flowmetry (Pen Flux System 5000, Perimed AB) in patients and controls. Skin

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Po-Chung Cheng Graduate Institute of Biomedical Sciences, China Medical University, Taichung, Taiwan

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Chia-Hung Kao Graduate Institute of Biomedical Sciences, China Medical University, Taichung, Taiwan

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prevention of diabetic CHD is an essential component of diabetes management guidelines ( 5 , 6 ). Diabetic dyslipidemia has several unique features. Insulin resistance increases the flux of free fatty acids to the liver, which leads to excessive plasma

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