66 research outputs found

    Nuclear Level Density and the Determination of Thermonuclear Rates for Astrophysics

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    The prediction of cross sections for nuclei far off stability is crucial in the field of nuclear astrophysics. We discuss the model mostly employed for such calculations: the statistical model (Hauser-Feshbach). Special emphasis is put on the uncertainties arising from nuclear level density descriptions and an improved global description is presented. Furthermore, criteria for the applicability of the statistical model are investigated and a "map" for the applicability of the model to reactions of stable and unstable nuclei with neutral and charged particles is given.Comment: REVTeX paper + 7 B/W figures + 2 color figures; PRC, in press. Also available at http://quasar.physik.unibas.ch/preps.htm

    DLG4-related synaptopathy: a new rare brain disorder

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    PURPOSE: Postsynaptic density protein-95 (PSD-95), encoded by DLG4, regulates excitatory synaptic function in the brain. Here we present the clinical and genetic features of 53 patients (42 previously unpublished) with DLG4 variants.METHODS: The clinical and genetic information were collected through GeneMatcher collaboration. All the individuals were investigated by local clinicians and the gene variants were identified by clinical exome/genome sequencing.RESULTS: The clinical picture was predominated by early onset global developmental delay, intellectual disability, autism spectrum disorder, and attention deficit-hyperactivity disorder, all of which point to a brain disorder. Marfanoid habitus, which was previously suggested to be a characteristic feature of DLG4-related phenotypes, was found in only nine individuals and despite some overlapping features, a distinct facial dysmorphism could not be established. Of the 45 different DLG4 variants, 39 were predicted to lead to loss of protein function and the majority occurred de novo (four with unknown origin). The six missense variants identified were suggested to lead to structural or functional changes by protein modeling studies.CONCLUSION: The present study shows that clinical manifestations associated with DLG4 overlap with those found in other neurodevelopmental disorders of synaptic dysfunction; thus, we designate this group of disorders as DLG4-related synaptopathy.Genetics of disease, diagnosis and treatmen

    Comparative trends and seasonal variation of 7Be, 210Pb and 137Cs at two altitude sites in the central part of France

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    The atmospheric concentrations of 137Cs, 210Pb, and 7Be were measured over a three-year period at two research stations located less than 12km apart and at different altitudes (puy de Dôme, 1465m a.s.l. and Opme, 660m a.s.l., France). Seasonal trends in all radionuclides were observed at both stations, with high concentration measured during the summer and low concentrations during the winter. The 210Pb concentrations at both stations were similar to each other. Higher concentrations of both 7Be and 137Cs were measured at puy de Dôme than at Opme. These observations can be explained by the stratospheric and upper tropospheric sources of 7Be and the long-range transportation of 137Cs at high altitudes. Air mass origins during sampling periods were classified into several groups by their route to the stations (marine, marine modified, continental and mediterranean). We observed that 7Be concentrations were constant regardless of the air mass origins, unlike 137Cs and 210Pb concentrations that increased when influenced by continental air masses. Higher 7Be concentrations were observed when air masses were arriving from the upper troposphere than from the boundary layer, the opposite was observed for 137Cs. The temporal trend in concentrations of 7Be shows good agreement with previous modelling studies suggesting that there is a good understanding of its sources and the atmospheric vertical mixing of this radionuclide. The sources and mixing of 210Pb, however, seem to be more complex than it appeared to be in previous modelling studies. © 2011 Elsevier Ltd

    A new method for assessing the aerosol to rain chemical composition relationships

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    International audienceMeasurements were conducted at three sampling sites located at different altitudes in the centre of France during two years, both in the rain and aerosol phases. The rain was sampled at a boundary layer site while the aerosol particles were collected at two different altitudes, which allow a better characterization of the vertical atmospheric column being washed out. Various chemical analyses were performed to characterize reactive (NO 3 -, SO 4 2-, NH 4 + and K +) and inert ( 7Be, 210Pb and 137Cs) species transfer from the aerosol to the rain phase. This set-up was ideal to calculate the washout ratio (W R) using different concentrations of the aerosol phase. Using the classical W R calculated with the aerosol concentration sampled at the same altitude than the rain collectors, we observed a seasonality of W R, with higher value in winter and lower value in summer for radionuclides. At the higher altitude site, local contaminations do not influence the aerosol concentration, which then should be representative of the whole atmospheric column. The annual variability is high at this site maybe because aerosol concentrations can be less concentrated than the whole atmospheric column when this later one is not well mixed. In order to increase the reliability of the W R, we propose a new method for calculating washout ratio from measurements at the rain collector level. This new calculation takes into account the height of the boundary layer, we observed that it decreased the variability of the washout ratio (for 7Be, 210Pb and NO 3 -), with less dependence to the season. © 2012 Elsevier B.V

    Three years of semicontinuous greenhouse gas measurements at the Puy de Dôme station (central France)

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    International audienceThree years of greenhouse gas measurements, obtained using a gas chromatograph (GC) system located at the Puy de Dôme station at 1465 m a.s.l. in central France, are presented. The GC system was installed in 2010 at Puy de Dôme and was designed for automatic and accurate semicontinuous measurements of atmospheric carbon dioxide, methane, nitrous oxide and sulfur hexafluoride mole fractions. We present in detail the instrumental setup and the calibration strategy, which together allow the GC to reach repeatabilities of 0.1 µmol mol −1 , 1.2 nmol mol −1 , 0.3 nmol mol −1 and 0.06 pmol mol −1 for CO2 , CH4 , N2O and SF6 , respectively. The analysis of the 3-year atmospheric time series revealed how the planetary boundary layer height drives the mole fractions observed at a mountain site such as Puy de Dôme where air masses alternate between the planetary boundary layer and the free troposphere. Accurate long-lived greenhouse gas measurements collo-cated with 222 Rn measurements as an atmospheric tracer allowed us to determine the CO2 , CH4 and N2O emissions in the catchment area of the station. The derived CO2 surface flux revealed a clear seasonal cycle, with net uptake by plant assimilation in the spring and net emission caused by the biosphere and burning of fossil fuel during the remainder of the year. We calculated a mean annual CO2 flux of 1310 ± 680 t CO2 km −2. The derived CH4 and N2O emissions in the station catchment area were 7.0 ± 4.0 t CH4 km −2 yr −1 and 1.8 ± 1.0 t N2O km −2 yr −1 , respectively. Our derived annual CH4 flux is in agreement with the national French inventory, whereas our derived N2O flux is 5 times larger than the same inventory
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