A mutation in X-linked inhibitor of apoptosis (G466X) leads to memory inflation of Epstein-Barr virus-specific T cells.

Mutations in the X-linked inhibitor of apoptosis (XIAP) gene have been associated with XLP-like disease, including recurrent Epstein-Barr virus (EBV)-related haemophagocytic lymphohystiocytosis (HLH), but the immunopathogenic bases of EBV-related disease in XIAP deficiency is unknown. We present the first analysis of EBV-specific T cell responses in functional XIAP deficiency. In a family of patients with a novel mutation in XIAP (G466X) leading to a late-truncated protein and varying clinical features, we identified gradual hypogammaglobulinaemia and large expansions of T cell subsets, including a prominent CD4(+) CD8(+) population. Extensive ex-vivo analyses showed that the expanded T cell subsets were dominated by EBV-specific cells with conserved cytotoxic, proliferative and interferon (IFN)-γ secretion capacity. The EBV load in blood fluctuated and was occasionally very high, indicating that the XIAP(G466X) mutation could impact upon EBV latency. XIAP deficiency may unravel a new immunopathogenic mechanism in EBV-associated disease.

Assigning a social status from face adornments: an fMRI study.

For at least 150,000 years, the human body has been culturally modified by the wearing of personal ornaments and probably by painting with red pigment. The present study used functional magnetic resonance imaging to explore the brain networks involved in attributing social status from face decorations. Results showed the fusiform gyrus, orbitofrontal cortex, and salience network were involved in social encoding, categorization, and evaluation. The hippocampus and parahippocampus were activated due to the memory and associative skills required for the task, while the inferior frontal gyrus likely interpreted face ornaments as symbols. Resting-state functional connectivity analysis clarified the interaction between these regions. The study highlights the importance of these neural interactions in the symbolic interpretation of social markers on the human face, which were likely active in early Homo species and intensified with Homo sapiens populations as more complex technologies were developed to culturalize the human face.

Reconstructing historical trends of Berre lagoon contamination from surface sediment datasets: influences of industrial regulations and anthropogenic silt inputs.

These last decades, the Berre lagoon (in southeastern France) has been deeply affected since the 1930s by strong inputs of contaminants associated with industrial development and since 1966 by huge inputs of freshwater and silts due to the installation of a hydroelectric power plant. Surveys of the surface sediment contamination have been sparsely performed since 1964 for management and research purposes. These surveys were performed by various laboratories that investigated different chemicals and sampling areas using different analysis protocols. Therefore, the available data are disconnected in time and space and differ in quality. In order to reconstruct coherent time series of sediment contamination from this heterogeneous datasets and to discuss the influences of industrial and hydroelectric discharges we used a statistical approach. This approach is based on Principal Component Analysis (PCA) and Fuzzy clustering analysis on data from one extensive survey realized on surface sediments in 1976. The PCA allowed identifying two geochemical indexes describing the main surface sediment geochemical characteristics. The fuzzy clustering analysis on these indexes allowed identifying sub-areas under the specific influence of industrial or hydroelectric discharges. This allowed us to reconstruct, for each sub-area, a coherent and interpretable long-term time series of sediment contamination from the available database. Reconstructed temporal trends allowed us to estimate: (i) the overall decrease of sediment contamination since the mid-1970 attributed to industrial discharge regulations enacted at this period and (ii) the dilution of the concentrations of sediment bound contaminants induced by the hydroelectric power plant and its associated particulate matter inputs.

Comparative structural and vibrational investigations between cocoa butter (CB) and cocoa butter equivalent (CBE) by ESI/MALDI-HRMS, XRD, DSC, MIR and Raman spectroscopy.

A high quality chocolate requires not only a shiny surface, a crunchy and pleasant texture, but also a proper resistance to blooming. All these characteristics are influenced by the physical and chemical properties of the components, which are directly related to their crystalline structure. Some works found that the proportion of cocoa butter (CB), cocoa butter equivalent (CBE) and milk fatty acid (AMF) tend to strongly delay the blooming when mixing them. The goal of our research is to determine how the choice of adding CBE to the mixture delays chocolate blooming. ESI/MALDI-HRMS, X-ray, DSC, MIR and Raman investigations were used to analyze the structure features and the vibrational modes of CB and CBE. The comparison of these experimental results between CB and CBE made it possible to highlight markers of differentiation between CB and CBE which seems to explain the impact of CBE in the chocolate blooming. Part of these triglycerides remains in form IV instead. The presence of the latter seems to be a key parameter that favors the transformation deceleration to the form VI, which is responsible for the fat bloom development.

Effects of ring diameter and wire tension on the axial biomechanics of four-ring circular external skeletal fixator constructs.

OBJECTIVE: To determine relative effects of ring diameter and wire tension on axial biomechanical properties of 4-ring circular external skeletal fixator constructs. SAMPLE POPULATION: 4-ring circular external skeletal fixator constructs and artificial bone models. PROCEDURE: 4-ring constructs were assembled, using 50-, 66-, 84-, or 118-mm-diameter rings. Two 1.6-mm-diameter fixation wires were attached to opposing surfaces of each ring at intersection angles of 90 degrees and placed through a gap-fracture bone model. Three examples of each construct were loaded in axial compression at 7 N/s to a maximum load of 400 N at each of 4 wire tensions (0, 30, 60, and 90 kg). Response variables were determined from resulting load-displacement curves (construct stiffness, load at 1 mm of displacement, displacement at 400 N). RESULTS: Ring diameter and wire tension had a significant effect on all response variables and had a significant interaction for construct stiffness and displacement at 400 N. Significant differences within all response variables were seen among all 4 ring diameters and all 4 wire tensions. As ring diameter increased, effect of increasing wire tension on gap stiffness and gap displacement at 400 N decreased. Ring diameter had a greater effect than wire tension on all response variables. CONCLUSIONS AND CLINICAL RELEVANCE: Although effects of wire tension decrease as ring diameter increases, placing tension on wires in larger ring constructs is important because these constructs are inherently less stiff. The differential contribution of ring diameter, wire tension, and their interactions must be considered when using circular external skeletal fixators.

Natural radionuclides (210)Po and (210)Pb in the Delaware and Chesapeake Estuaries: modeling scavenging rates and residence times.

During the spring and summer months of 2012, (210)Po and (210)Pb activity were measured in the dissolved and particulate phases from the Delaware and upper Chesapeake estuaries. The upper Delaware estuary, near the freshwater end member, was characterized by high-suspended matter concentrations that scavenged dissolved (210)Po and (210)Pb. Box models were applied using mass balance calculations to assess the nuclides residence times in each estuary. Only 60% of the dissolved (210)Po and 55% of the dissolved (210)Pb from the Delaware estuary were exported to coastal waters. A large fraction of soluble (210)Po and (210)Pb within the estuary was either reversibly adsorbed onto suspended particles, trapped in sediment accumulation zones (such as intertidal marshes), bioaccumulated into phytoplankton and discharged to the coastal ocean. The upper Chesapeake estuary was largely characterized by sub-oxic bottom waters that contained higher concentrations of dissolved (210)Po and (210)Pb, hypothesized to be subjected to redox cycling of manganese. The Delaware and Chesapeake estuary mean residence times for (210)Po differed significantly at 86 ± 7 and 126 ± 10 days respectively, while they were similar for (210)Pb (67 ± 6-55 ± 5 days). The difference in residence times corresponds to the greater extent of biogeochemical scavenging and regeneration processes within the upper Chesapeake.

Mobility of Cd and Cu in formulated sediments coated with iron hydroxides and/or humic acids: a DGT and DGT-PROFS modeling approach.

The diffusive gradients technique in thin films (DGT) was used to investigate the kinetic resupply of Cd and Cu to pore water from the solid phase. For the sake of simplification, experiments were performed using formulated sediments that differed in the presence or absence of humic acids (HA) and/or of iron hydroxides (i.e., goethite and ferrihydrite). The effects of the time after the contamination of the solid phase (aging effect) on formulated sediments that were coated with goethite and HA and spiked with Cd were also evaluated. Kinetic DGT results were interpreted using the newly developed, multi-compartmental model DGT-PROFS. Due to Cu humate formation, the addition of HA slightly increased the Cu concentration in the pore water independent of the effect of the iron hydroxide coating on the formulated sediments and slightly decreased that of Cd. The impact of 8-190d of aging resulted in a significant decrease in the Cd concentration of the pore water over an increasing incubation time. Modeling our results with DGT-PROFS led to the following conclusions concerning the impact of HA and iron hydroxides on Cd and Cu availability. First, in the presence of HA and absence of iron hydroxides, Cd is associated mainly with weak sites, while Cu is bound to strong sites. Similarly, in the presence of both iron hydroxides and HA, Cu appeared to be more heavily associated with the strong sites than did Cd. When the incubation time increased from 8 to 190d, a proportion of Cd initially adsorbed onto weak sites transferred to the strong sites, suggesting that the adsorption of Cd on sediments is controlled partially by slow kinetic processes.