Search Results - "Debray, F"

Diagnostic pitfall in antenatal manifestations of CPT II deficiency by Boemer, F., Deberg, M., Schoos, R., Caberg, J.-H., Gaillez, S., Dugauquier, C., Delbecque, K., François, A., Maton, P., Demonceau, N., Senterre, G., Ferdinandusse, S., Debray, F.-G.

“…Carnitine palmitoyltransferase II (CPT2) deficiency is a rare inborn error of mitochondrial fatty acid metabolism associated with various phenotypes. Whereas…”
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A next-generation newborn screening pilot study: NGS on dried blood spots detects causal mutations in patients with inherited metabolic diseases by Boemer, F., Fasquelle, C., d’Otreppe, S., Josse, C., Dideberg, V., Segers, K., Guissard, V., Capraro, V., Debray, FG, Bours, V.

“…The range of applications performed on dried blood spots (DBS) widely broadened during the past decades to now include next-generation sequencing (NGS)…”
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Effects of high static magnetic field on distribution of solid particles in BiZn immiscible alloys with metastable miscibility gap by Zheng, T.X., Zhong, Y.B., Lei, Z.S., Ren, W.L., Ren, Z.M., Debray, F., Beaugnon, E., Fautrelle, Y.

“…•Immiscible alloys without any segregation were manufactured under 29T of HSMF.•Stokes and Marangoni movement led to the segregation under HSMF lower than…”
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Magnets for a Muon Collider-Needs and Plans by Bottura, L., Accettura, C., Amemiya, N., Auchmann, B., Berg, J.S., Bersani, A., Bertarelli, A., Boattini, F., Bordini, B., Borges de Sousa, P., Breschi, M., Caiffi, B., Chaud, X., Debray, F., Dudarev, A., Eisterer, M., Fabbri, S., Farinon, S., Ferracin, P., De Gersem, H., Kario, A., Kolehmainen, A., Kosse, J., Lorenzo Gomez, J., Losito, R., Mariotto, S., Mentink, M., Mulder, T., Musenich, R., Novelli, D., Ogitsu, T., Palmer, M., Pavan, J., Piekarz, H., Portone, A., Quettier, L., Rochepault, E., Rossi, L., Salmi, T., Schneider-Muntau, H., Senatore, C., Statera, M., Ten Kate, H.H.J., Testoni, P., Vallone, G., Verweij, A., Van Weelderen, R., Wozniak, M., Yamamoto, A., Yang, Y., Zhai, Y., Zlobin, A.

“…We describe the magnet challenges for a Muon Collider, an exciting option considered for the future of particle physics at the energy frontier. Starting from…”
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DC High Field Magnets at the LNCMI by Debray, F., Dumas, J., Trophime, C., Vidal, N.

“…The Laboratoire National des Champs Magnétiques Intenses (LNCMI) is a French large scale facility enabling researchers to perform experiments in the highest…”
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The French High Magnetic Field Facility by Béard, J., Debray, F.

“…The Laboratoire National des Champs Magnétiques Intenses (LNCMI) is a host laboratory for experiments in high magnetic fields. It was created on the 1st of…”
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Design Study of a 10-T REBCO Insert Solenoid by Borgnolutti, F., Badel, A., Benkel, T., Chaud, X., Debray, F., Fazilleau, P., Lecrevisse, T., Tixador, P.

“…As a first step to develop HTS insert technology, we plan to build a 10-T REBCO solenoid that will be tested at the French National High Magnetic Field…”
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Transient Recording System for Power Supply and High Field Magnet Fault Analysis: Its Use for Magnet Aging Surveillance and Accident Diagnosis by Sala, Ph, Daversin, C., Debray, F., Dumas, J., Trophime, C.

“…The Laboratoire National des Champs Magnétiques Intenses, which is a member of the European Magnetic Field Laboratory, is the French facility enabling…”
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Hepatocyte transplantation using the domino concept in a child with tetrabiopterin nonresponsive phenylketonuria by Stéphenne, X, Debray, F G, Smets, F, Jazouli, N, Sana, G, Tondreau, T, Menten, R, Goffette, P, Boemer, F, Schoos, R, Gersting, S W, Najimi, M, Muntau, A C, Goyens, P, Sokal, E M

“…Phenylketonuria is a metabolic disease caused by phenylalanine hydroxylase deficiency. Treatment is based on a strict natural protein-restricted diet that is…”
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Pyruvate dehydrogenase deficiency presenting as intermittent isolated acute ataxia by Debray, F-G, Lambert, M, Gagne, R, Maranda, B, Laframboise, R, MacKay, N, Robinson, B H, Mitchell, G A

“…The aim of this study is to report and emphasize unusual presentations of pyruvate dehydrogenase (PDH) deficiency (OMIM 312170). PDH activity and PDHA1 gene…”
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Reduced brain choline in homocystinuria due to remethylation defects by DEBRAY, F.-G, BOULANGER, Y, BLOM, H. J, JAKOBS, C, LEVY, E, MITCHELL, G. A, LAMBERT, M, KHIAT, A, DECARIE, J.-C, ORQUIN, J, ROY, M.-S, LORTIE, A, RAMOS, F, VERHOEVEN, N. M, STRUYS, E

“…To investigate whether secondary impairment of the transmethylation pathway is a mechanism underlying the neurologic involvement in homocystinuria due to…”
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Quench Propagation in YBCO Pancake: Experimental and Computational Results by Lecrevisse, T., Chaud, X., Debray, F., Devaux, M., Fazilleau, P., Juster, F. P., Miyoshi, Y., Rey, J.-M, Tixador, P., Vincent, B.

“…High-temperature superconductors are promising materials for future applications such as high field magnets thanks to their ability to carry high current…”
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Influence of the static high magnetic field on the liquid–liquid phase separation during solidifying the hyper-monotectic alloys by Wang, J., Zhong, Y. B., Fautrelle, Y., Zheng, T. X., Li, F., Ren, Z. M., Debray, F.

“…Magnetic in-situ quenching refers to fixing and quenching the sample at a static high magnetic field (SHMF) up to 18 T; it has been achieved by a specially…”
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High intensity neutrino oscillation facilities in Europe by Edgecock, T. R., Caretta, O., Davenne, T., Densam, C., Fitton, M., Kelliher, D., Loveridge, P., Machida, S., Prior, C., Rogers, C., Rooney, M., Thomason, J., Wilcox, D., Wildner, E., Efthymiopoulos, I., Garoby, R., Gilardoni, S., Hansen, C., Benedetto, E., Jensen, E., Kosmicki, A., Martini, M., Osborne, J., Prior, G., Stora, T., Melo Mendonca, T., Vlachoudis, V., Waaijer, C., Cupial, P., Chancé, A., Longhin, A., Payet, J., Zito, M., Baussan, E., Bobeth, C., Bouquerel, E., Dracos, M., Gaudiot, G., Lepers, B., Osswald, F., Poussot, P., Vassilopoulos, N., Wurtz, J., Zeter, V., Bielski, J., Kozien, M., Lacny, L., Skoczen, B., Szybinski, B., Ustrycka, A., Wroblewski, A., Marie-Jeanne, M., Balint, P., Fourel, C., Giraud, J., Jacob, J., Lamy, T., Latrasse, L., Sortais, P., Thuillier, T., Mitrofanov, S., Loiselet, M., Keutgen, Th, Delbar, Th, Debray, F., Trophine, C., Veys, S., Daversin, C., Zorin, V., Izotov, I., Skalyga, V., Burt, G., Dexter, A. C., Kravchuk, V. L., Marchi, T., Cinausero, M., Gramegna, F., De Angelis, G., Prete, G., Collazuol, G., Laveder, M., Mazzocco, M., Mezzetto, M., Signorini, C., Vardaci, E., Di Nitto, A., Brondi, A., La Rana, G., Migliozzi, P., Moro, R., Palladino, V., Gelli, N., Berkovits, D., Hass, M., Hirsh, T. Y., Schaumann, M., Stahl, A., Wehner, J., Bross, A., Kopp, J.

“…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…”
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Status of the EuCARD 5.4-T REBCO Dipole Magnet by Borgnolutti, F., Durante, M., Debray, F., Rifflet, J.-M, De Rijk, G., Tixador, P., Tudela, J.-M

“…A 5.4-T REBCO insert dipole magnet is being built at CEA Saclay to study the viability of hybrid accelerator magnets made of high- and low-temperature…”
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Status of the 43-T Hybrid Magnet of LNCMI-Grenoble by Pugnat, P., Manil, P., Molinie, F., Pes, C., Pfister, R., Queinec, Y., Pissard, M., Ronayette, L., Trophime, C., Vincent, B., Barbier, R., Berriaud, C., Berthier, R., Caplanne, G., Debray, F., Fazilleau, P., Hanoux, P., Hervieu, B.

“…Based on a close collaboration between CEA and CNRS, a new hybrid magnet is being built at LNCMI-Grenoble. By combining a resistive insert, which is made of…”
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Superconducting and Mechanical Properties of Impregnated REBCO Pancake Coils Under Large Hoop Stress by Awaji, S., Oguro, H., Suwa, T., Suzuki, T., Watanabe, K., Nishijima, G., Hanai, S., Marukawa, K., Daibo, M., Saito, T., Sakamoto, H., Inoue, I., Miyoshi, Y., Chaud, X., Debray, F., Tixador, P.

“…We performed hoop stress tests of REBCO multilayer pancake coils impregnated by epoxy resin. The mechanical deformation and electric field-current properties…”
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Progress Report on the 43 T Hybrid Magnet of the LNCMI-Grenoble by Pugnat, P., Barbier, R., Berriaud, C., Berthier, R., Debray, F., Fazilleau, P., Hervieu, B., Manil, P., Massinger, M., Pes, C., Pfister, R., Pissard, M., Ronayette, L., Trophime, C.

“…A CEA-CNRS French collaboration is currently developing a new hybrid magnet to produce in a first step a continuous magnetic field of 43 T in a 34-mm warm bore…”
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Phenotypic variability among patients with hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome homozygous for the delF188 mutation in SLC25A15 by Debray, F-G, Lambert, M, Lemieux, B, Soucy, J F, Drouin, R, Fenyves, D, Dubé, J, Maranda, B, Laframboise, R, Mitchell, G A

“…Hyperornithinaemia-hyperammonaemia-homocitrullinuria (HHH) syndrome (OMIM 238970) is caused by impaired ornithine transport across the inner mitochondrial…”
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HTS Dipole Insert Developments by Rey, J.-M, Devaux, M., Bertinelli, F., Chaud, X., Debray, F., Durante, M., Favre, G., Fazilleau, P., Lecrevisse, T., Mayri, C., Pes, C., Pottier, F., Sorbi, M., Stenvall, A., Tixador, P., Tudela, J., Tardy, T., Volpini, G.

“…Future accelerator magnets will need to reach a magnetic field in the 20 T range. Reaching such a magnetic field is a challenge only reachable using high…”
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