Calibration of quasi-static aberrations in exoplanet direct-imaging instruments with a Zernike phase-mask sensor. II. Concept validation with ZELDA on VLT/SPHERE

Warm or massive gas giant planets, brown dwarfs, and debris disks around nearby stars are now routinely observed by dedicated high-contrast imaging instruments on large, ground-based observatories. These facilities include extreme adaptive optics (ExAO) and state-of-the-art coronagraphy to achieve unprecedented sensitivities for exoplanet detection and spectral characterization. However, differential aberrations between the ExAO sensing path and the science path represent a critical limitation for the detection of giant planets with a contrast lower than a few $10^{-6}$ at very small separations (<0.3\as) from their host star. In our previous work, we proposed a wavefront sensor based on Zernike phase contrast methods to circumvent this issue and measure these quasi-static aberrations at a nanometric level. We present the design, manufacturing and testing of ZELDA, a prototype that was installed on VLT/SPHERE during its reintegration in Chile. Using the internal light source of the instrument, we performed measurements in the presence of Zernike or Fourier modes introduced with the deformable mirror. Our experimental and simulation results are consistent, confirming the ability of our sensor to measure small aberrations (<50 nm rms) with nanometric accuracy. We then corrected the long-lived non-common path aberrations in SPHERE based on ZELDA measurements. We estimated a contrast gain of 10 in the coronagraphic image at 0.2\as, reaching the raw contrast limit set by the coronagraph in the instrument. The simplicity of the design and its phase reconstruction algorithm makes ZELDA an excellent candidate for the on-line measurements of quasi-static aberrations during the observations. The implementation of a ZELDA-based sensing path on the current and future facilities (ELTs, future space missions) could ease the observation of the cold gaseous or massive rocky planets around nearby stars.Comment: 13 pages, 12 figures, A&A accepted on June 3rd, 2016. v2 after language editin

The Cerenkov effect revisited: from swimming ducks to zero modes in gravitational analogs

We present an interdisciplinary review of the generalized Cerenkov emission of radiation from uniformly moving sources in the different contexts of classical electromagnetism, superfluid hydrodynamics, and classical hydrodynamics. The details of each specific physical systems enter our theory via the dispersion law of the excitations. A geometrical recipe to obtain the emission patterns in both real and wavevector space from the geometrical shape of the dispersion law is discussed and applied to a number of cases of current experimental interest. Some consequences of these emission processes onto the stability of condensed-matter analogs of gravitational systems are finally illustrated.Comment: Lecture Notes at the IX SIGRAV School on "Analogue Gravity" in Como, Italy from May 16th-21th, 201

Deletion of the GABAA α2-subunit does not alter self dministration of cocaine or reinstatement of cocaine seeking

Rationale GABAA receptors containing α2-subunits are highly represented in brain areas that are involved in motivation and reward, and have been associated with addiction to several drugs, including cocaine. We have shown previously that a deletion of the α2-subunit results in an absence of sensitisation to cocaine. Objective We investigated the reinforcing properties of cocaine in GABAA α2-subunit knockout (KO) mice using an intravenous self-administration procedure. Methods α2-subunit wildtype (WT), heterozygous (HT) and KO mice were trained to lever press for a 30 % condensed milk solution. After implantation with a jugular catheter, mice were trained to lever press for cocaine (0.5 mg/kg/infusion) during ten daily sessions. Responding was extinguished and the mice tested for cue- and cocaine-primed reinstatement. Separate groups of mice were trained to respond for decreasing doses of cocaine (0.25, 0.125, 0.06 and 0.03 mg/kg). Results No differences were found in acquisition of lever pressing for milk. All genotypes acquired self-administration of cocaine and did not differ in rates of self-administration, dose dependency or reinstatement. However, whilst WT and HT mice showed a dose-dependent increase in lever pressing during the cue presentation, KO mice did not. Conclusions Despite a reported absence of sensitisation, motivation to obtain cocaine remains unchanged in KO and HT mice. Reinstatement of cocaine seeking by cocaine and cocaine-paired cues is also unaffected. We postulate that whilst not directly involved in reward perception, the α2-subunit may be involved in modulating the “energising” aspect of cocaine’s effects on reward-seeking

The GUINEVERE Project for Accelerator Driven System Physics

paper 9414International audienceThe GUINEVERE project is part of the EUROTRANS Integrated Project of the 6th EURATOM Framework Programme. It is mainly devoted to ADS on-line reactivity monitoring validation, sub-criticality determination and operational procedures (loading, start-up, shut-down, ...) as a follow-up of the MUSE experiments. The project consists in coupling a fast lead core, set-up in the VENUS reactor at SCK*CEN Mol (B), with a GENEPI neutron source under construction by CNRS. To accommodate the accelerator in a vertical coupling configuration, the VENUS building is being heightened. The fast core will be loaded with enriched Uranium and will be moderated and reflected with solid lead (zero power experiment). For the purpose of the experimental programme, the neutron source has to be operated not only in pulsed mode but also in continuous mode to investigate the current-to-flux reactivity indicator in representative conditions of a powerful ADS. In this latter mode it is also required to make short beam interruptions to have access to the neutron population decrease as a function of time: from this spectrum it will be possible to apply different analysis techniques such as "prompt decay" fitting techniques and "source jerk" techniques. Beam interruptions will be repeated at a programmable frequency to improve time spectra statistics. Different sub-criticality levels (keff=0.99, 0.97, 0.95, ...) will be investigated in order to obtain a full set of data points for the final overall validation of the methodology. This paper describes the status of the experimental facility assembling, and the foreseen experimental programme to be started

Spondyloenchondrodysplasia Due to Mutations in ACP5: A Comprehensive Survey

Purpose: Spondyloenchondrodysplasia is a rare immuno-osseous dysplasia caused by biallelic mutations in ACP5. We aimed to provide a survey of the skeletal, neurological and immune manifestations of this disease in a cohort of molecularly confirmed cases. Methods: We compiled clinical, genetic and serological data from a total of 26 patients from 18 pedigrees, all with biallelic ACP5 mutations. Results: We observed a variability in skeletal, neurological and immune phenotypes, which was sometimes marked even between affected siblings. In total, 22 of 26 patients manifested autoimmune disease, most frequently autoimmune thrombocytopenia and systemic lupus erythematosus. Four patients were considered to demonstrate no clinical autoimmune disease, although two were positive for autoantibodies. In the majority of patients tested we detected upregulated expression of interferon-stimulated genes (ISGs), in keeping with the autoimmune phenotype and the likely immune-regulatory function of the deficient protein tartrate resistant acid phosphatase (TRAP). Two mutation positive patients did not demonstrate an upregulation of ISGs, including one patient with significant autoimmune disease controlled by immunosuppressive therapy. Conclusions: Our data expand the known phenotype of SPENCD. We propose that the OMIM differentiation between spondyloenchondrodysplasia and spondyloenchondrodysplasia with immune dysregulation is no longer appropriate, since the molecular evidence that we provide suggests that these phenotypes represent a continuum of the same disorder. In addition, the absence of an interferon signature following immunomodulatory treatments in a patient with significant autoimmune disease may indicate a therapeutic response important for the immune manifestations of spondyloenchondrodysplasia

Morphine activation of mu opioid receptors causes disinhibition of neurons in the ventral tegmental area mediated by β-arrestin2 and c-Src

Abstract The tyrosine kinase, c-Src, participates in mu opioid receptor (MOP) mediated inhibition in sensory neurons in which β-arrestin2 (β-arr2) is implicated in its recruitment. Mice lacking β-arr2 exhibit increased sensitivity to morphine reinforcement; however, whether β-arr2 and/or c-Src participate in the actions of opioids in neurons within the reward pathway is unknown. It is also unclear whether morphine acts exclusively through MOPs, or involves delta opioid receptors (DOPs). We examined the involvement of MOPs, DOPs, β-arr2 and c-Src in the inhibition by morphine of GABAergic inhibitory postsynaptic currents (IPSCs) recorded from neurons in the mouse ventral tegmental area. Morphine inhibited spontaneous IPSC frequency, mainly through MOPs, with only a negligible effect remaining in MOP−/− neurons. However, a reduction in the inhibition by morphine for DOP−/− c.f. WT neurons and a DPDPE-induced decrease of IPSC frequency revealed a role for DOPs. The application of the c-Src inhibitor, PP2, to WT neurons also reduced inhibition by morphine, while the inactive PP3, and the MEK inhibitor, SL327, had no effect. Inhibition of IPSC frequency by morphine was also reduced in β-arr2−/− neurons in which PP2 caused no further reduction. These data suggest that inhibition of IPSCs by morphine involves a β-arr2/c-Src mediated mechanism

The GUINEVERE project at the VENUS facility

Proc. on CD Rom log315International audienceThe GUINEVERE project is an international project in the framework of IP-EUROTRANS, the FP6 program which aims at addressing the main issues for ADS development in the framework of partitioning and transmutation for nuclear waste volume and radiotoxicity reduction. The GUINEVERE project is carried out in the context of domain 2 of IP-EUROTRANS, ECATS, devoted to specific experiments for the coupling of an accelerator, a target and a subcritical core. These experiments should provide an answer to the questions of online reactivity monitoring, sub-criticality determination and operational procedures (loading, start-up, shutdown, …) in an ADS by 2009-2010. The project has the objective to couple a fast lead core, within the VENUS building operated by the SCK•CEN, with a neutron generator able to work in three different modes: pulsed, continuous and continuous with beam interruptions at the millisecond scale. In order to achieve this goal, the VENUS facility has to be adapted and a modified GENEPI-3C accelerator has to be designed and constructed. The paper describes the main modifications to the reactor core and facility and to the accelerator, which will be executed during the years 2008 and 2009, and the experimental programme which will start in 2009

A patient with an uncommon complication from insertion of a central venous catheter: A case report

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Clinical reappraisal of SHORT syndrome with PIK3R1 mutations: towards recommendation for molecular testing and management

International audienceSHORT syndrome has historically been defined by its acronym: short stature (S), hyperextensibility of joints and/or inguinal hernia (H), ocular depression (O), Rieger abnormality (R) and teething delay (T). More recently several research groups have identified PIK3R1 mutations as responsible for SHORT syndrome. Knowledge of the molecular etiology of SHORT syndrome has permitted a reassessment of the clinical phenotype. The detailed phenotypes of 32 individuals with SHORT syndrome and PIK3R1 mutation, including eight newly ascertained individuals, were studied to fully define the syndrome and the indications for PIK3R1 testing. The major features described in the SHORT acronym were not universally seen and only half (52%) had 4 or more of the classic features. The commonly observed clinical features of SHORT syndrome seen in the cohort included IUGR \textless 10(th) percentile, postnatal growth restriction, lipoatrophy and the characteristic facial gestalt. Anterior chamber defects and insulin resistance or diabetes were also observed but were not as prevalent. The less specific, or minor features of SHORT syndrome include teething delay, thin wrinkled skin, speech delay, sensorineural deafness, hyperextensibility of joints and inguinal hernia. Given the high risk of diabetes mellitus, regular monitoring of glucose metabolism is warranted. An echocardiogram, ophthalmological and hearing assessments are also recommended