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The study of nuclear fission is encountering renewed interest with the development of GEN-IV reactor concepts, mostly working in the neutron fast energy domain. To support the fast reactor technologies, new high quality nuclear data are needed. New facilities are being constructed to produce high intensity neutron beams from hundreds of keV to few tens of MeV (Licorne, NFS, nELBE, ...). They will open new opportunities to provide nuclear data. In this framework the development of an experimental setup called FALSTAFF for a characterisation of actinide fission fragments has been undertaken. Fission fragment yields and associated neutron multiplicities will be measured as a function of the neutron energy. Based on time-of-flight and residual energy technique, the setup will allow the simultaneous measurement of the complementary fragment velocity and energy. The FALSTAFF setup and the upgrade of the first arm prototype with the new ionisation chamber CALIBER will be presented. The performances of the experimental apparatus is discussed.

In the past years, the fission studies have been mainly focused on thermal fission because most of the current nuclear reactors work in this energy domain. With the development of GEN-IV reactor concepts, mainly working in the fast energy domain, new nuclear data are needed. The FALSTAFF spectrometer under development at CEA-Saclay, France, is a two-arm spectrometer which will provide mass yields before (2V method) and after (EV method) neutron evaporation and consequently will have access to the neutron multiplicity as a function of mass. The axial ionization chamber, in addition to the kinetic energy value, will measure the energy loss profile of the fragment along its track. This energy loss profile will give information about the fragment nuclear charge. This paper will focus on recent developments on the FALSTAFF design. A special attention will be paid to the impact of the detector material thickness on the uncertainty of different observables.

The steadily increasing luminosity of the LHC requires an upgrade with high-rate and high-resolution detector technology for the inner end cap of the ATLAS muon spectrometer: the New Small Wheels (NSW). In order to achieve the goal of precision tracking at a hit rate of about 15 kHz/cm\\(^2\\) at the inner radius of the NSW, large area Micromegas quadruplets with 100\\, spatial resolution per plane have been produced. % IRFU, from the CEA research center of Saclay, is responsible for the production and validation of LM1 Micromegas modules. The construction, production, qualification and validation of the largest Micromegas detectors ever built are reported here. Performance results under cosmic muon characterisation will also be discussed.

The STEREO experiment is a very short baseline reactor antineutrino experiment aiming at testing the hypothesis of light sterile neutrinos as an explanation of the deficit of the observed neutrino interaction rate with respect to the predicted rate, known as the Reactor Antineutrino Anomaly. The detector center is located 10 m away from the compact, highly \\(^{235}\\)U enriched core of the research nuclear reactor of the Institut Laue Langevin in Grenoble, France. This paper describes the STEREO site, the detector components and associated shielding designed to suppress the external sources of background which were characterized on site. It reports the performances in terms of detector response and energy reconstruction.

A 10 kilo-tonne dual-phase liquid argon TPC is one of the detector options considered for the Deep Underground Neutrino Experiment (DUNE). The detector technology relies on amplification of the ionisation charge in ultra-pure argon vapour and oers several advantages compared to the traditional single-phase liquid argon TPCs. A 4.2 tonne dual-phase liquid argon TPC prototype, the largest of its kind, with an active volume of 3x1x1 \\(m^3\\) has been constructed and operated at CERN. In this paper we describe in detail the experimental setup and detector components as well as report on the operation experience. We also present the first results on the achieved charge amplification, prompt scintillation and electroluminescence detection, and purity of the liquid argon from analyses of a collected sample of cosmic ray muons.

Originally designed as a new nuclear reactor monitoring device, the Nucifer detector has successfully detected its first neutrinos. We provide the second shortest baseline measurement of the reactor neutrino flux. The detection of electron antineutrinos emitted in the decay chains of the fission products, combined with reactor core simulations, provides an new tool to assess both the thermal power and the fissile content of the whole nuclear core and could be used by the Inter- national Agency for Atomic Energy (IAEA) to enhance the Safeguards of civil nuclear reactors. Deployed at only 7.2m away from the compact Osiris research reactor core (70MW) operating at the Saclay research centre of the French Alternative Energies and Atomic Energy Commission (CEA), the experiment also exhibits a well-suited configuration to search for a new short baseline oscillation. We report the first results of the Nucifer experiment, describing the performances of the 0.85m3 detector remotely operating at a shallow depth equivalent to 12m of water and under intense background radiation conditions. Based on 145 (106) days of data with reactor ON (OFF), leading to the detection of an estimated 40760 electron antineutrinos, the mean number of detected antineutrinos is 281 +- 7(stat) +- 18(syst) electron antineutrinos/day, in agreement with the prediction 277(23) electron antineutrinos/day. Due the the large background no conclusive results on the existence of light sterile neutrinos could be derived, however. As a first societal application we quantify how antineutrinos could be used for the Plutonium Management and Disposition Agreement.

This article quickly summarizes the performances and results of the GOLF/SoHO resonant spectrometer, thus justifying to go a step further. We then recall the characteristics of the multichannel resonant GOLF-NG spectrometer and present the first successful performances of the laboratory tests on the prototype and also the limitations of this first technological instrument. Scientific questions and an observation strategy are discussed.

The primary challenge of GOLF-NG (Global Oscillations at Low Frequency New Generation) is the detection of the low-frequency solar gravity and acoustic modes, as well as the possibility to measure the high-frequency chromospheric modes. On June 8th 2008, the first sunlight observations with the multichannel resonant GOLF-NG prototype spectrometer were obtained at the Observatorio del Teide (Tenerife). The instrument performs integrated (Sun-as-a-star), Doppler velocity measurements, simultaneously at eight different heights in the D1 sodium line profile, corresponding to photospheric and chromospheric layers of the solar atmosphere. In order to study its performances, to validate the conceived strategy, and to estimate the necessary improvements, this prototype has been running on a daily basis over the whole summer of 2008 at the Observatorio del Teide. We present here the results of the first GOLF-NG observations, clearly showing the characteristics of the 5-minute oscillatory signal at different heights in the solar atmosphere. We compare these signals with simultaneous observations from GOLF/SOHO and from the Mark-I instrument -- a node of the BiSON network, operating at the same site.

Hypercalciuria is a major cause of nephrolithiasis, and is a common and complex disorder involving genetic and environmental factors. Identification of genetic factors for monogenic forms of hypercalciuria is hampered by the limited availability of large families, and to facilitate such studies, we screened for hypercalciuria in mice from an N-ethyl-N-nitrosourea mutagenesis programme. We identified a mouse with autosomal dominant hypercalciuria (HCALC1). Linkage studies mapped the Hcalc1 locus to a 11.94 Mb region on chromosome 6 containing the transient receptor potential cation channel, subfamily V, members 5 (Trpv5) and 6 (Trpv6) genes. DNA sequence analysis of coding regions, intron-exon boundaries and promoters of Trpv5 and Trpv6 identified a novel T to C transition in codon 682 of TRPV5, mutating a conserved serine to a proline (S682P). Compared to wild-type littermates, heterozygous (Trpv5(682P/+)) and homozygous (Trpv5(682P/682P)) mutant mice had hypercalciuria, polyuria, hyperphosphaturia and a more acidic urine, and ∼10% of males developed tubulointerstitial nephritis. Trpv5(682P/682P) mice also had normal plasma parathyroid hormone but increased 1,25-dihydroxyvitamin D(3) concentrations without increased bone resorption, consistent with a renal defect for the hypercalciuria. Expression of the S682P mutation in human embryonic kidney cells revealed that TRPV5-S682P-expressing cells had a lower baseline intracellular calcium concentration than wild-type TRPV5-expressing cells, suggesting an altered calcium permeability. Immunohistological studies revealed a selective decrease in TRPV5-expression from the renal distal convoluted tubules of Trpv5(682P/+) and Trpv5(682P/682P) mice consistent with a trafficking defect. In addition, Trpv5(682P/682P) mice had a reduction in renal expression of the intracellular calcium-binding protein, calbindin-D(28K), consistent with a specific defect in TRPV5-mediated renal calcium reabsorption. Thus, our findings indicate that the TRPV5 S682P mutant is functionally significant and study of HCALC1, a novel model for autosomal dominant hypercalciuria, may help further our understanding of renal calcium reabsorption and hypercalciuria.

Non-syndromic kyphosis is a common disorder that is associated with significant morbidity and has a strong genetic involvement; however, the causative genes remain to be identified, as such studies are hampered by genetic heterogeneity, small families and various modes of inheritance. To overcome these limitations, we investigated 12 week old progeny of mice treated with the chemical mutagen N-ethyl-N-nitrosourea (ENU) using phenotypic assessments including dysmorphology, radiography, and dual-energy X-ray absorptiometry. This identified a mouse with autosomal recessive kyphosis (KYLB). KYLB mice, when compared to unaffected littermates, had: thoraco-lumbar kyphosis, larger vertebrae, and increased body length and increased bone area. In addition, female KYLB mice had increases in bone mineral content and plasma alkaline phosphatase activity. Recombination mapping localized the Kylb locus to a 5.5Mb region on chromosome 15A1, which contained 51 genes, including the natriuretic peptide receptor 3 (Npr3) gene. DNA sequence analysis of Npr3 identified a missense mutation, Tyr209Asn, which introduced an N-linked glycosylation consensus sequence. Expression of wild-type NPR3 and the KYLB-associated Tyr209Asn NPR3 mutant in COS-7 cells demonstrated the mutant to be associated with abnormal N-linked glycosylation and retention in the endoplasmic reticulum that resulted in its absence from the plasma membrane. NPR3 is a decoy receptor for C-type natriuretic peptide (CNP), which also binds to NPR2 and stimulates mitogen-activated protein kinase (MAPK) signaling, thereby increasing the number and size of hypertrophic chondrocytes. Histomorphometric analysis of KYLB vertebrae and tibiae showed delayed endochondral ossification and expansion of the hypertrophic zones of the growth plates, and immunohistochemistry revealed increased p38 MAPK phosphorylation throughout the growth plates of KYLB vertebrae. Thus, we established a model of kyphosis due to a novel NPR3 mutation, in which loss of plasma membrane NPR3 expression results in increased MAPK pathway activation, causing elongation of the vertebrae and resulting in kyphosis.