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C L Bodinham, L Smith, E L Thomas, J D Bell, J R Swann, A Costabile, D Russell-Jones, A M Umpleby and M D Robertson

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

Open access

Nicolai Preisler, Pascal Laforêt, Karen Lindhardt Madsen, Edith Husu, Christoffer Rasmus Vissing, Gitte Hedermann, Henrik Galbo, Christopher Lindberg and John Vissing

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.

Open access

Ashley N Reeb, Andrea Ziegler and Reigh-Yi Lin

-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

Open access

Aasem Saif, Shrook Mousa, Maha Assem, Nashwa Tharwat and Alaa Abdelhamid

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

Open access

Wang Chengji and Fan Xianjin

vascular inflammation and increased proinflammatory cytokines ( 35 ). In diabetes, PKC is activated by advanced glycation end (AGE) products and polyol pathway flux ( 36 ). Also, chronic hyperglycemia stimulates synthesis of DAG and activates DAG

Open access

Peng Fan, Chao-Xia Lu, Di Zhang, Kun-Qi Yang, Pei-Pei Lu, Ying Zhang, Xu Meng, Su-Fang Hao, Fang Luo, Ya-Xin Liu, Hui-Min Zhang, Lei Song, Jun Cai, Xue Zhang and Xian-Liang Zhou

reabsorption by paracellular flux and potassium secretion by the renal outer medullary K + channels ( 13 ). Three homologous subunits share 30–40% identity, and each one comprises an extracellular loop, two transmembrane domains and a short intracellular N

Open access

Sayaka Kawano, Yukiko Kawagoe, Kenji Kuwasako, Satoshi Shimamoto, Koji Igarashi, Mariko Tokashiki, Kazuo Kitamura and Johji Kato

Nakao K Minami T Yamada C Ueshima K . The effects of super-flux (high performance) dialyzer on plasma glycosylated pro-B-type natriuretic peptide (proBNP) and glycosylated N-terminal proBNP in end-stage renal disease patients on dialysis

Open access

Michaela Keuper

reported previously, indicating higher glycolytic fluxes, impaired conversion of lactate to pyruvate and/or impaired pyruvate import into the mitochondria ( 1 , 112 , 113 ). This is in line with suggestions on the increased requirement of glycolytic

Open access

Fernanda A Correa, Ericka B Trarbach, Cintia Tusset, Ana Claudia Latronico, Luciana R Montenegro, Luciani R Carvalho, Marcela M Franca, Aline P Otto, Everlayny F Costalonga, Vinicius N Brito, Ana Paula Abreu, Mirian Y Nishi, Alexander A L Jorge, Ivo J P Arnhold, Yisrael Sidis, Nelly Pitteloud and Berenice B Mendonca

is located within the last intracellular loop, and results of functional analyses revealed normal transcription activity in an Erg-Luc assay but decreased calcium mobilization activity (72% of WT) in an aequorin-based Ca 2+ flux assay. Its expression

Open access

Lili Liu, Zhuo Shao, Ying Xia, Jiabi Qin, Yang Xiao, Zhiguang Zhou and Zubing Mei

Honkanen-Scott M Shaw JAM Lovat PE Arden C. Glucagon-like peptide 1 protects pancreatic beta-cells from death by increasing autophagic flux and restoring lysosomal function . Diabetes 2017 66 1272 – 1285 . ( https://doi.org/10.2337/db16-1009 ) 10