TY  - JOUR
A1  - Edgecock, T. R.
A1  - Caretta, O.
A1  - Davenne, T.
A1  - Densam, C.
A1  - Fitton, M.
A1  - Kelliher, D.
A1  - Loveridge, P.
A1  - Machida, S.
A1  - Prior, C.
A1  - Rogers, C.
A1  - Rooney, M.
A1  - Thomason, J.
A1  - Wilcox, D.
A1  - Wildner, E.
A1  - Efthymiopoulos, I.
A1  - Garoby, R.
A1  - Gilardoni, S.
A1  - Hansen, C.
A1  - Benedetto, E.
A1  - Jensen, E.
A1  - Kosmicki, A.
A1  - Martini, M.
A1  - Osborne, J.
A1  - Prior, G.
A1  - Stora, T.
A1  - Melo Mendonca, T.
A1  - Vlachoudis, V.
A1  - Waaijer, C.
A1  - Cupial, P.
A1  - Chancé, A.
A1  - Longhin, A.
A1  - Payet, J.
A1  - Zito, M.
A1  - Baussan, E.
A1  - Bobeth, C.
A1  - Bouquerel, E.
A1  - Dracos, M.
A1  - Gaudiot, G.
A1  - Lepers, B.
A1  - Osswald, F.
A1  - Poussot, P.
A1  - Vassilopoulos, N.
A1  - Wurtz, J.
A1  - Zeter, V.
A1  - Bielski, J.
A1  - Kozien, M.
A1  - Lacny, L.
A1  - Skoczen, B.
A1  - Szybinski, B.
A1  - Ustrycka, A.
A1  - Wroblewski, A.
A1  - Marie-Jeanne, M.
A1  - Balint, P.
A1  - Fourel, C.
A1  - Giraud, J.
A1  - Jacob, J.
A1  - Lamy, T.
A1  - Latrasse, L.
A1  - Sortais, P.
A1  - Thuillier, T.
A1  - Mitrofanov, S.
A1  - Loiselet, M.
A1  - Keutgen, Th.
A1  - Delbar, Th.
A1  - Debray, F.
A1  - Trophine, C.
A1  - Veys, S.
A1  - Daversin, C.
A1  - Zorin, V.
A1  - Izotov, I.
A1  - Skalyga, V.
A1  - Burt, G.
A1  - Dexter, A. C.
A1  - Kravchuk, V. L.
A1  - Marchi, T.
A1  - Cinausero, M.
A1  - Gramegna, F.
A1  - De Angelis, G.
A1  - Prete, G.
A1  - Collazuol, G.
A1  - Laveder, M.
A1  - Mazzocco, M.
A1  - Mezzetto, M.
A1  - Signorini, C.
A1  - Vardaci, E.
A1  - Di Nitto, A.
A1  - Brondi, A.
A1  - La Rana, G.
A1  - Migliozzi, P.
A1  - Moro, R.
A1  - Palladino, V.
A1  - Gelli, N.
A1  - Berkovits, D.
A1  - Hass, M.
A1  - Hirsh, T. Y.
A1  - Schuhmann, M.
A1  - Stahl, A.
A1  - Wehner, J.
A1  - Bross, A.
A1  - Kopp, J.
A1  - Neuffer, D.
A1  - Wands, R.
A1  - Bayes, R.
A1  - Laing, A.
A1  - Soler, P.
A1  - Agarwalla, S. K.
A1  - Cervera Villanueva, A.
A1  - Donini, A.
A1  - Ghosh, T.
A1  - Gómez Cadenas, J. J.
A1  - Hernández, P.
A1  - Martín-Albo, J.
A1  - Mena, O.
A1  - Burguet-Castell, J.
A1  - Agostino, L.
A1  - Buizza-Avanzini, M.
A1  - Marafini, M.
A1  - Patzak, T.
A1  - Tonazzo, A.
A1  - Duchesneau, D.
A1  - Mosca, L.
A1  - Bogomilov, M.
A1  - Karadzhov, Y.
A1  - Matev, R.
A1  - Tsenov, R.
A1  - Akhmedov, E.
A1  - Blennow, M.
A1  - Lindner, M.
A1  - Schwetz, T.
A1  - Fernández Martinez, E.
A1  - Maltoni, M.
A1  - Menéndez, J.
A1  - Giunti, C.
A1  - González García, M. C.
A1  - Salvado, J.
A1  - Coloma, P.
A1  - Huber, P.
A1  - Li, T.
A1  - López Pavón, J.
A1  - Orme, C.
A1  - Pascoli, S.
A1  - Meloni, D.
A1  - Tang, J.
A1  - Winter, W.
A1  - Ohlsson, T.
A1  - Zhang, H.
A1  - Scotto-Lavina, L.
A1  - Terranova, F.
A1  - Bonesini, M.
A1  - Tortora, L.
A1  - Alekou, A.
A1  - Aslaninejad, M.
A1  - Bontoiu, C.
A1  - Kurup, A.
A1  - Jenner, L. J.
A1  - Long, K.
A1  - Pasternak, J.
A1  - Pozimski, J.
A1  - Back, J. J.
A1  - Harrison, P.
A1  - Beard, K.
A1  - Bogacz, A.
A1  - Berg, J. S.
A1  - Stratakis, D.
A1  - Witte, H.
A1  - Snopok, P.
A1  - Bliss, N.
A1  - Cordwell, M.
A1  - Moss, A.
A1  - Pattalwar, S.
A1  - Apollonio, M.
T1  - High intensity neutrino oscillation facilities in Europe
JF  - Physical Review Special Topics-Accelerators and Beams
N2  - The EUROnu project has studied three possible options for future, high intensity neutrino oscillation facilities in Europe. The first is a Super Beam, in which the neutrinos come from the decay of pions created by bombarding targets with a 4 MW proton beam from the CERN High Power Superconducting Proton Linac. The far detector for this facility is the 500 kt MEMPHYS water Cherenkov, located in the Frejus tunnel. The second facility is the Neutrino Factory, in which the neutrinos come from the decay of mu(+) and mu(-) beams in a storage ring. The far detector in this case is a 100 kt magnetized iron neutrino detector at a baseline of 2000 km. The third option is a Beta Beam, in which the neutrinos come from the decay of beta emitting isotopes, in particular He-6 and Ne-18, also stored in a ring. The far detector is also the MEMPHYS detector in the Frejus tunnel. EUROnu has undertaken conceptual designs of these facilities and studied the performance of the detectors. Based on this, it has determined the physics reach of each facility, in particular for the measurement of CP violation in the lepton sector, and estimated the cost of construction. These have demonstrated that the best facility to build is the Neutrino Factory. However, if a powerful proton driver is constructed for another purpose or if the MEMPHYS detector is built for astroparticle physics, the Super Beam also becomes very attractive.
KW  - EMMA
KW  - beta-beam
Y1  - 2013
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-126611
VL  - 16
IS  - 2
ER  - 
TY  - JOUR
A1  - Schade, A.
A1  - Bader, A.
A1  - Huber, T.
A1  - Kuhn, S.
A1  - Czyszanowski, T.
A1  - Pfenning, A.
A1  - Rygała, M.
A1  - Smołka, T.
A1  - Motyka, M.
A1  - Sęk, G.
A1  - Hartmann, F.
A1  - Höfling, S.
T1  - Monolithic high contrast grating on GaSb/AlAsSb based epitaxial structures for mid-infrared wavelength applications
JF  - Optics Express
N2  - We demonstrate monolithic high contrast gratings (MHCG) based on GaSb/AlAs0.08Sb0.92 epitaxial structures with sub-wavelength gratings enabling high reflection of unpolarized mid-infrared radiation at the wavelength range from 2.5 to 5 µm. We study the reflectivity wavelength dependence of MHCGs with ridge widths ranging from 220 to 984 nm and fixed 2.6 µm grating period and demonstrate that peak reflectivity of above 0.7 can be shifted from 3.0 to 4.3 µm for ridge widths from 220 to 984 nm, respectively. Maximum reflectivity of up to 0.9 at 4 µm can be achieved. The experiments are in good agreement with numerical simulations, confirming high process flexibility in terms of peak reflectivity and wavelength selection. MHCGs have hitherto been regarded as mirrors enabling high reflection of selected light polarization. With this work, we show that thoughtfully designed MHCG yields high reflectivity for both orthogonal polarizations simultaneously. Our experiment demonstrates that MHCGs are promising candidates to replace conventional mirrors like distributed Bragg reflectors to realize resonator based optical and optoelectronic devices such as resonant cavity enhanced light emitting diodes and resonant cavity enhanced photodetectors in the mid-infrared spectral region, for which epitaxial growth of distributed Bragg reflectors is challenging.
KW  - monolithic grating
KW  - high contrast
KW  - mid-infrared wavelength applications
KW  - epitaxial structures
KW  - GaSb/AlAsSb
Y1  - 2023
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-350346
VL  - 31
IS  - 10
ER  - 
TY  - JOUR
A1  - Kotte, K.
A1  - Löw, F.
A1  - Huber, S. G.
A1  - Krause, T.
A1  - Mulder, I.
A1  - Schöler, H. F.
T1  - Organohalogen emissions from saline environments - spatial extrapolation using remote sensing as most promising tool
JF  - Biogeosciences
N2  - Due to their negative water budget most recent semi-/arid regions are characterized by vast evaporates (salt lakes and salty soils). We recently identified those hyper-saline environments as additional sources for a multitude of volatile halogenated organohalogens (VOX). These compounds can affect the ozone layer of the stratosphere and play a key role in the production of aerosols. A remote sensing based analysis was performed in the Southern Aral Sea basin, providing information of major soil types as well as their extent and spatial and temporal evolution. VOX production has been determined in dry and moist soil samples after 24 h. Several C1- and C2 organohalogens have been found in hyper-saline topsoil profiles, including CH3Cl, CH3Br, CHBr3 and CHCl3. The range of organohalogens also includes trans-1,2-dichloroethene (DCE), which is reported here to be produced naturally for the first time. Using MODIS time series and supervised image classification a daily production rate for DCE has been calculated for the 15 000 km\(^2\) ranging research area in the southern Aralkum. The applied laboratory setup simulates a short-term change in climatic conditions, starting from dried-out saline soil that is instantly humidified during rain events or flooding. It describes the general VOX production potential, but allows only for a rough estimation of resulting emission loads. VOX emissions are expected to increase in the future since the area of salt affected soils is expanding due to the regressing Aral Sea. Opportunities, limits and requirements of satellite based rapid change detection and salt classification are discussed.
KW  - aral sea basin
KW  - methyl-bromide
KW  - methane emissions
KW  - abiotic formation
KW  - time series
KW  - salt lakes
KW  - land
KW  - Uzbekistan
KW  - soils/sediments
KW  - classifiaction
Y1  - 2012
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-134265
VL  - 9
IS  - 3
ER  - 
TY  - JOUR
A1  - Dubail, Johanne
A1  - Huber, Céline
A1  - Chantepie, Sandrine
A1  - Sonntag, Stephan
A1  - Tüysüz, Beyhan
A1  - Mihci, Ercan
A1  - Gordon, Christopher T.
A1  - Steichen-Gersdorf, Elisabeth
A1  - Amiel, Jeanne
A1  - Nur, Banu
A1  - Stolte-Dijkstra, Irene
A1  - van Eerde, Albertien M.
A1  - van Gassen, Koen L.
A1  - Breugem, Corstiaan C.
A1  - Stegmann, Alexander
A1  - Lekszas, Caroline
A1  - Maroofian, Reza
A1  - Karimiani, Ehsan Ghayoor
A1  - Bruneel, Arnaud
A1  - Seta, Nathalie
A1  - Munnich, Arnold
A1  - Papy-Garcia, Dulce
A1  - De La Dure-Molla, Muriel
A1  - Cormier-Daire, Valérie
T1  - SLC10A7 mutations cause a skeletal dysplasia with amelogenesis imperfecta mediated by GAG biosynthesis defects
JF  - Nature Communications
N2  - Skeletal dysplasia with multiple dislocations are severe disorders characterized by dislocations of large joints and short stature. The majority of them have been linked to pathogenic variants in genes encoding glycosyltransferases, sulfotransferases or epimerases required for glycosaminoglycan synthesis. Using exome sequencing, we identify homozygous mutations in SLC10A7 in six individuals with skeletal dysplasia with multiple dislocations and amelogenesis imperfecta. SLC10A7 encodes a 10-transmembrane-domain transporter located at the plasma membrane. Functional studies in vitro demonstrate that SLC10A7 mutations reduce SLC10A7 protein expression. We generate a Slc10a7−/− mouse model, which displays shortened long bones, growth plate disorganization and tooth enamel anomalies, recapitulating the human phenotype. Furthermore, we identify decreased heparan sulfate levels in Slc10a7−/− mouse cartilage and patient fibroblasts. Finally, we find an abnormal N-glycoprotein electrophoretic profile in patient blood samples. Together, our findings support the involvement of SLC10A7 in glycosaminoglycan synthesis and specifically in skeletal development.
KW  - bone development
KW  - disease genetics
KW  - medical genetics
Y1  - 2018
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-226377
VL  - 9
ER  -