74 research outputs found
TROP 03 - Le paludisme en Guyane Française, deux populations : l'une liée à l'activité d'orpaillage et celle des peuples autochtones
National audienceLe paludisme en Guyane Française, deux populations : l'une liée à l'activité d'orpaillage et celle des peuples autochtone
Potentialité d'invasion, en Méditerranée occidentale, par des potyvirus proches du Papaya ringspot virus (PRSV)
Supplément 1National audienc
Technical feasibility assessment of a PEM fuel cell refrigerator system
PEM Fuel Cells (PEMFCs), fueled by hydrogen, are electrochemical devices that convert hydrogen to useful power and two by-products: heat and water. They cover an important part of power applications namely in the transportation area, and in other practical applications that are either stationary or portable. In particular, the domestic refrigerator is one of the daily and indispensable applications but with a high-energy demand due to the high running time cycles. This work is a technical assessment of the feasibility of building a coupled âPEM Fuel Cell â Refrigeratorâ system. Real technical data for the refrigerator are collected, processed and evaluated. The obtained results show reasonable flows consumption rates. In fact, the refrigerator requires a flow rate of 1.607 slpm of hydrogen and 8 slpm of air at a pressure of respectively 3 atm and 1 atm. The water is produced at a rate of 1.285 10â3 slpm. The annual amount of hydrogen consumed by the refrigerator is estimated to 28, 47 kg. The energy provided to the refrigerator is about 130 W and the energy needed by the air compressor is 28, 24 W. A technical solution is suggested at the end of this work to reduce the start and stop cycles of the fuel cell
Technical feasibility assessment of a PEM fuel cell refrigerator system
International audienc
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Targeting RyR Activity Boosts Antisense Exon 44 and 45 Skipping in Human DMD Skeletal or Cardiac Muscle Culture Models.
Systemic delivery of antisense oligonucleotides (AO) for DMD exon skipping has proven effective for reframing DMD mRNA, rescuing dystrophin expression, and slowing disease progression in animal models. In humans with Duchenne muscular dystrophy treated with AOs, low levels of dystrophin have been induced, and modest slowing of disease progression has been observed, highlighting the need for improved efficiency of human skipping drugs. Here, we demonstrate that dantrolene and Rycals S107 and ARM210 potentiate AO-mediated exon skipping of exon 44 or exon 45 in patient-derived myotube cultures with appropriate mutations. Further, dantrolene is shown to boost AO-mediated exon skipping in patient-derived, induced cardiomyocyte cultures. Our findings further validate the ryanodine receptors (RyR) as the likely target responsible for exon skip boosting and demonstrate potential applicability beyond exon 51 skipping. These data provide preclinical support of dantrolene trial as an adjuvant to AO-mediated exon-skipping therapy in humans and identify a novel Rycal, ARM210, for development as a potential exon-skipping booster. Further, they highlight the value of mutation-specific DMD culture models for basic discovery, preclinical drug screening and translation of personalized genetic medicines
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Large in-frame 5' deletions in DMD associated with mild Duchenne muscular dystrophy: Two case reports and a review of the literature.
Duchenne muscular dystrophy is caused by mutations in the dystrophin-encoding DMD gene. While Duchenne is most commonly caused by large intragenic deletions that cause frameshift and complete loss of dystrophin expression, in-frame deletions in DMD can result in the expression of internally truncated dystrophin proteins and may be associated with a milder phenotype. In this study, we describe two individuals with large in-frame 5' deletions (exon 3-23 and exon 3-28) that remove the majority of the N-terminal region, including part of the actin binding and central rod domains. Both patients had progressive muscle weakness during childhood but are observed to have a relatively mild disease course compared to typical Duchenne. We show that in muscle biopsies from both patients, truncated dystrophin is expressed at the sarcolemma. We have additionally developed a patient-specific fibroblast-derived cell model, which can be inducibly reprogrammed to form myotubes that largely recapitulate biopsy findings for the patient with the exon 3-23 deletion, providing a culture model for future investigation of this unusual case. We discuss these mutations in the context of previously reported 5' in-frame DMD deletions and relevant animal models, and review the spectrum of phenotypes associated with these deletions
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