Statistical interpolation of ozone measurements from satellite data (TOMS, SBUV and SAGE II) using the kriging method

International audienceThis study demonstrates that ordinary kriging in spherical coordinates using experimental semi-variograms provides highly usable results, especially near the pole in winter and/or where there could be data missing over large areas. In addition, kriging allows display of the spatial variability of daily ozone measurements at different pressure levels. Three satellite data sets were used: Total Ozone Mapping Spectrometer (TOMS) data, Solar Backscattered UltraViolet (SBUV), and the Stratospheric Aerosol and Gas Experiment (SAGE II) ozone profiles. Since SBUV is a nadir-viewing instrument, measurements are only taken along the sun-synchronous polar orbits of the satellite. SAGE II is a limb-viewing solar occultation instrument, and measurements have high vertical resolution but poor daily coverage. TOMS has wider coverage with equidistant distribution of data (resolution 1° × 1.25°) but provides no vertical information. Comparisons of the resulting SBUV-interpolated (column-integrated) ozone field with TOMS data are strongly in agreement, with a global correlation of close to 98%. Comparisons of SBUV-interpolated ozone profiles with daily SAGE II profiles are relatively good, and comparable to those found in the literature. The interpolated ozone layers at different pressure levels are shown

Data assimilation of Soil Moisture and Ocean Salinity (SMOS) observations into the Mercator Ocean operational system: focus on the El Niño 2015 event

Monitoring sea surface salinity (SSS) is important for understanding and forecasting the ocean circulation. It is even crucial in the context of the intensification of the water cycle. Until recently, SSS was one of the less observed essential ocean variables. Only sparse in situ observations, mostly closer to 5&thinsp;m depth than the surface, were available to estimate the SSS. The recent satellite ESA Soil Moisture and Ocean Salinity (SMOS), NASA Aquarius SAC-D and Soil Moisture Active Passive (SMAP) missions have made it possible for the first time to measure SSS from space and can bring a valuable additional constraint to control the model salinity. Nevertheless, satellite SSS still contains some residual biases that must be removed prior to bias correction and data assimilation. One of the major challenges of this study is to estimate the SSS bias and a suitable observation error for the data assimilation system. It was made possible by modifying a 3D-Var bias correction scheme and by using the analysis of the residuals and errors with an adapted statistical technique. This article presents the design and the analysis of an observing system experiment (OSE) conducted with the 0.25∘ resolution Mercator Ocean global analysis and forecasting system during the El Niño 2015/16 event. The SSS data assimilation constrains the model to be closer to the near-surface salinity observations in a coherent way with the other data sets already routinely assimilated in an operational context. This also shows that the overestimation of E–P is corrected by data assimilation through salting in regions where precipitations are higher. Globally, the SMOS SSS assimilation has a positive impact in salinity over the top 30&thinsp;m. Comparisons to independent salinity data sets show a small but positive impact and corroborate the fact that the impact of SMOS SSS assimilation is larger in the Intertropical Convergence Zone (ITCZ) and South Pacific Convergence Zone (SPCZ) regions. There is little impact on the sea surface temperature (SST) and sea surface height (SSH) error statistics. Nevertheless, the SSH seems to be impacted by the tropical instability wave (TIW) propagation, itself linked to changes in barrier layer thickness (BLT). Finally, this study helped us to progress in the understanding of the biases and errors that can degrade the SMOS SSS data assimilation performance.</p

Ataxia with oculomotor apraxia type 2: clinical, biological and genotype/phenotype correlation study of a cohort of 90 patients

Ataxia with oculomotor apraxia type 2 (AOA2) is an autosomal recessive disease due to mutations in the senataxin gene, causing progressive cerebellar ataxia with peripheral neuropathy, cerebellar atrophy, occasional oculomotor apraxia and elevated alpha-feto-protein (AFP) serum level. We compiled a series of 67 previously reported and 58 novel ataxic patients who underwent senataxin gene sequencing because of suspected AOA2. An AOA2 diagnosis was established for 90 patients, originating from 15 countries worldwide, and 25 new senataxin gene mutations were found. In patients with AOA2, median AFP serum level was 31.0 mu g/l at diagnosis, which was higher than the median AFP level of AOA2 negative patients: 13.8 mu g/l, P = 0.0004; itself higher than the normal level (3.4 mu g/l, range from 0.5 to 17.2 mu g/l) because elevated AFP was one of the possible selection criteria. Polyneuropathy was found in 97.5% of AOA2 patients, cerebellar atrophy in 96%, occasional oculomotor apraxia in 51%, pyramidal signs in 20.5%, head tremor in 14%, dystonia in 13.5%, strabismus in 12.3% and chorea in 9.5%. No patient was lacking both peripheral neuropathy and cerebellar atrophy. The age at onset and presence of occasional oculomotor apraxia were negatively correlated to the progression rate of the disease (P = 0.03 and P = 0.009, respectively), whereas strabismus was positively correlated to the progression rate (P = 0.03). An increased AFP level as well as cerebellar atrophy seem to be stable in the course of the disease and to occur mostly at or before the onset of the disease. One of the two patients with a normal AFP level at diagnosis had high AFP levels 4 years later, while the other had borderline levels. The probability of missing AOA2 diagnosis, in case of sequencing senataxin gene only in non-Friedreich ataxia non-ataxia-telangiectasia ataxic patients with AFP level >= 7 mu g/l, is 0.23% and the probability for a non-Friedreich ataxia non-ataxia-telangiectasia ataxic patient to be affected with AOA2 with AFP levels >= 7 mu g/l is 46%. Therefore, selection of patients with an AFP level above 7 mu g/l for senataxin gene sequencing is a good strategy for AOA2 diagnosis. Pyramidal signs and dystonia were more frequent and disease was less severe with missense mutations in the helicase domain of senataxin gene than with missense mutations out of helicase domain and deletion and nonsense mutations (P = 0.001, P = 0.008 and P = 0.01, respectively). The lack of pyramidal signs in most patients may be explained by masking due to severe motor neuropathy

Feasibility and Performance of the Staged Z-Pinch: A One-dimensional Study with FLASH and MACH2

Z-pinch platforms constitute a promising pathway to fusion energy research. Here, we present a one-dimensional numerical study of the staged Z-pinch (SZP) concept using the FLASH and MACH2 codes. We discuss the verification of the codes using two analytical benchmarks that include Z-pinch-relevant physics, building confidence on the codes' ability to model such experiments. Then, FLASH is used to simulate two different SZP configurations: a xenon gas-puff liner (SZP1*) and a silver solid liner (SZP2). The SZP2 results are compared against previously published MACH2 results, and a new code-to-code comparison on SZP1* is presented. Using an ideal equation of state and analytical transport coefficients, FLASH yields a fuel convergence ratio (CR) of approximately 39 and a mass-averaged fuel ion temperature slightly below 1 keV for the SZP2 scheme, significantly lower than the full-physics MACH2 prediction. For the new SZP1* configuration, full-physics FLASH simulations furnish large and inherently unstable CRs (> 300), but achieve fuel ion temperatures of many keV. While MACH2 also predicts high temperatures, the fuel stagnates at a smaller CR. The integrated code-to-code comparison reveals how magnetic insulation, heat conduction, and radiation transport affect platform performance and the feasibility of the SZP concept

Recessive Ataxia Diagnosis Algorithm for the Next Generation Sequencing Era

OBJECTIVE: Differential diagnosis of autosomal recessive cerebellar ataxias can be challenging. A ranking algorithm named RADIAL that predicts the molecular diagnosis based on the clinical phenotype of a patient has been developed to guide genetic testing and to align genetic findings with the clinical context. METHODS: An algorithm that follows clinical practice, including patient history, clinical, magnetic resonance imaging, electromyography, and biomarker features, was developed following a review of the literature on 67 autosomal recessive cerebellar ataxias and personal clinical experience. Frequency and specificity of each feature were defined for each autosomal recessive cerebellar ataxia, and corresponding prediction scores were assigned. Clinical and paraclinical features of patients are entered into the algorithm, and a patient's total score for each autosomal recessive cerebellar ataxia is calculated, producing a ranking of possible diagnoses. Sensitivity and specificity of the algorithm were assessed by blinded analysis of a multinational cohort of 834 patients with molecularly confirmed autosomal recessive cerebellar ataxia. The performance of the algorithm was assessed versus a blinded panel of autosomal recessive cerebellar ataxia experts. RESULTS: The correct diagnosis was ranked within the top 3 highest‐scoring diagnoses at a sensitivity and specificity of >90% for 84% and 91% of the evaluated genes, respectively. Mean sensitivity and specificity of the top 3 highest‐scoring diagnoses were 92% and 95%, respectively. The algorithm outperformed the panel of ataxia experts (p = 0.001). INTERPRETATION: Our algorithm is highly sensitive and specific, accurately predicting the underlying molecular diagnoses of autosomal recessive cerebellar ataxias, thereby guiding targeted sequencing or facilitating interpretation of next‐generation sequencing data

New Trends in Beverage Packaging Systems: A Review

New trends in beverage packaging are focusing on the structure modification of packaging materials and the development of new active and/or intelligent systems, which can interact with the product or its environment, improving the conservation of beverages, such as wine, juice or beer, customer acceptability, and food security. In this paper, the main nutritional and organoleptic degradation processes of beverages, such as oxidative degradation or changes in the aromatic profiles, which influence their color and volatile composition are summarized. Finally, the description of the current situation of beverage packaging materials and new possible, emerging strategies to overcome some of the pending issues are discussed

Tau, prions and Aβ: the triad of neurodegeneration

This article highlights the features that connect prion diseases with other cerebral amyloidoses and how these relate to neurodegeneration, with focus on tau phosphorylation. It also discusses similarities between prion disease and Alzheimer’s disease: mechanisms of amyloid formation, neurotoxicity, pathways involved in triggering tau phosphorylation, links to cell cycle pathways and neuronal apoptosis. We review previous evidence of prion diseases triggering hyperphosphorylation of tau, and complement these findings with cases from our collection of genetic, sporadic and transmitted forms of prion diseases. This includes the novel finding that tau phosphorylation consistently occurs in sporadic CJD, in the absence of amyloid plaques

The coffee genome provides insight into the convergent evolution of caffeine biosynthesis.

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Mitochondrial oxodicarboxylate carrier deficiency is associated with mitochondrial DNA depletion and spinal muscular atrophy-like disease.

PURPOSE: To understand the role of the mitochondrial oxodicarboxylate carrier (SLC25A21) in the development of spinal muscular atrophy-like disease. METHODS: We identified a novel pathogenic variant in a patient by whole-exome sequencing. The pathogenicity of the mutation was studied by transport assays, computer modeling, followed by targeted metabolic testing and in vitro studies in human fibroblasts and neurons. RESULTS: The patient carries a homozygous pathogenic variant c.695A>G; p.(Lys232Arg) in the SLC25A21 gene, encoding the mitochondrial oxodicarboxylate carrier, and developed spinal muscular atrophy and mitochondrial myopathy. Transport assays show that the mutation renders SLC25A21 dysfunctional and 2-oxoadipate cannot be imported into the mitochondrial matrix. Computer models of central metabolism predicted that impaired transport of oxodicarboxylate disrupts the pathways of lysine and tryptophan degradation, and causes accumulation of 2-oxoadipate, pipecolic acid, and quinolinic acid, which was confirmed in the patient's urine by targeted metabolomics. Exposure to 2-oxoadipate and quinolinic acid decreased the level of mitochondrial complexes in neuronal cells (SH-SY5Y) and induced apoptosis. CONCLUSION: Mitochondrial oxodicarboxylate carrier deficiency leads to mitochondrial dysfunction and the accumulation of oxoadipate and quinolinic acid, which in turn cause toxicity in spinal motor neurons leading to spinal muscular atrophy-like disease