Room temperature ionic liquids based on cationic porphyrin derivatives and tetrakis(pentafluorophenyl)borate anion

A series of 11 low melting ionic liquids based on meso-substituted A(3)B-porphyrins and A(2)B(2)-porphyrins containing one or two pyridyl substituents have been synthesized in high yields. Three of them are liquids at room temperature. All these porphyrinic salts were characterized by H-1 NMR, F-19 NMR, MALDI-TOF mass spectrometry, elemental analysis and UV-visible spectroscopy. The thermal properties and conductivity values of these salt derivatives have been also measured. A specific conductivity value of up to 4 mS.cm(-1) could be obtained for a compound having the counter-anion B(C6F5)(4)(-

A mutation of the human EPHB2 gene leads to a major platelet functional defect.

The ephrin transmembrane receptor family of tyrosine kinases is involved in platelet function. We report the first EPHB2 variant affecting platelets in 2 siblings (P1 and P2) from a consanguineous family with recurrent bleeding and normal platelet counts. Whole-exome sequencing identified a c.2233C>T variant (missense p.R745C) of the EPHB2 gene. P1 and P2 were homozygous for this variant, while their asymptomatic parents were heterozygous. The p.R745C variant within the tyrosine kinase domain was associated with defects in platelet aggregation, αIIbβ3 activation, and granule secretion induced by G-protein-coupled receptor (GPCR) agonists and convulxin, as well as in thrombus formation on collagen under flow. In contrast, clot retraction, flow-dependent platelet adhesion, and spreading on fibrinogen were only mildly affected, indicating limited effects on αIIbβ3 outside-in signaling. Most importantly, Lyn, Syk, and FcRγ phosphorylation, the initial steps in glycoprotein VI (GPVI) platelet signaling were drastically impaired in the absence of platelet-platelet contact, indicating a positive role for EPHB2 in GPVI activation. Likewise platelet activation by PAR4-AP showed defective Src activation, as opposed to normal protein kinase C activity and Ca2+ mobilization. Overexpression of wild-type and R745C EPHB2 variant in RBL-2H3 (rat basophilic leukemia) cells stably expressing human GPVI confirmed that EPHB2 R745C mutation impaired EPHB2 autophosphorylation but had no effect on ephrin ligand-induced EPHB2 clustering, suggesting it did not interfere with EPHB2-ephrin-mediated cell-to-cell contact. In conclusion, this novel inherited platelet disorder affecting EPHB2 demonstrates this tyrosine kinase receptor plays an important role in platelet function through crosstalk with GPVI and GPCR signaling

Formation and Dissolution of Hydride Precipitates in Zirconium Alloys: Crystallographic Orientation Relationships and Stability after Temperature Cycling

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Hydride Precipitates in Zirconium Alloys: Evolution of Dissolution and Precipitation Temperatures During Thermal Cycling Correlated to Microstructure Features

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THE C70 ARRONAX AND BEAM LINES STATUS

International audienceThe C70 ARRONAX is a high intensity (up to 2x375uA) multi-particle cyclotron aiming at R&D on material and radiolysis and production of radioisotopes. It began its hands-on phase in December 2010, and is currently undergoing beam lines' modification in experimental halls for present and future experiments. Characterisation of the beams at the end of beamlines is of particular importance to determine the capacity of the cyclotron for end-line experimental users. A program of beam characterisation is being performed based in one hand on dedicated diagnostics e.g. beam profilers, faraday cups, alumina foils and in the other hand on a series of Geant4 beam simulations. The out coming results of the measurements, along with the simulations, are detailed in this report for proton and alpha particle beams, as well as the future prospects of the characterisation program

Mitogen and Stress-activated Kinase-1 deficiency and transcriptional dysregulation in Huntington's Disease

Collaborative Congress of the European-Society-of-Gene-and-Cell-Therapy/French-Society-of-Cell-and-Gen e-Therapy, Versailles, FRANCE, OCT 25-29, 2012International audienceno abstrac

Evaluation of Sodium Boiling Models Using KNS-37 Loss of Flow Experiments

International audienceAbstract The computational codes used in the evaluation of the European sodium fast reactor—safety measures assessment and research tools(ESFR-SMART) reactor performance and specifically their sodium boiling models are assessed using two KNS-37 loss of flow (LOF) experiments, i.e., L22 and L29 tests, where boiling onset and two-phase flow regime up to dry-out occurred. The well-equipped KNS-37 experimental facility provided very valuable information for understanding the physical phenomena occurring in a 37-pin subassembly under LOF conditions, as well as experimental data to be used for computational tools validation. NATOF-2D, SAS-SFR, TRACE, ASTEC-Na, CATHARE-2, CATHARE-3, and NEPTUNE_CFD codes have been used in this exercise in order to compare the various boiling models and conclude on the advantages and limitations of them based on the comparison against the experimental data. Beyond boiling onset, the various sodium two-phase flow approaches determine the ability of the code to correctly represent the rewetting and voiding phases as well as cladding dry-out onset. A simulation performed by a computational fluid dynamics (CFD) approach (NEPTUNE_CFD code) taking into account liquid–vapor interfaces by an interface-tracking method is also shown and compared with the others approaches. Conclusions on each code performance are presented where the improvements needed to solve the issues encountered are included. This paper provides a first step in the process to investigate the required evaluation of the sodium two-phase flow models able to assess the safety of new SFR core designs (e.g., low void cores) under accidental conditions such as unprotected loss of flow (ULOF) transients

Improving silicon nitride ring resonator performances on 300 mm industrial environment for point of care applications (Conference Presentation)

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