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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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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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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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-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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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
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
Faculty of Medicine, University of Oslo, Oslo, Norway
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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
Department of Endocrinology, Zunyi Medical University, Zunyi, 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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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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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
