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See detailPhenotypic variation of a novel nonsense mutation in the P0 intracellular domain
Senderek, J.; Ramaekers, Vincent ULg; Zerres, K. et al

in Journal of the Neurological Sciences (2001), 192(1-2), 49-51

Mutations in the gene for the peripheral myelin protein zero (P0, MPZ) cause type 1B of Charcot-Marie-Tooth sensorimotor neuropathy (CMT1B). Here we report a German family with a novel heterozygous P0 ... [more ▼]

Mutations in the gene for the peripheral myelin protein zero (P0, MPZ) cause type 1B of Charcot-Marie-Tooth sensorimotor neuropathy (CMT1B). Here we report a German family with a novel heterozygous P0 nonsense mutation (G206X) that supposedly removes four-fifths of the amino acid residues constituting the P0 intracellular domain. The 12-year-old propositus had childhood-onset CMT1B associated with bilateral pes cavus, moderate lower limb weakness, and mildly reduced sensory qualities in the distal legs. The electrophysiology was consistent with a demyelinating neuropathy. He inherited the mutation from his mother who had no complaints but slight pes cavus deformity and slow nerve conduction velocities (NCV). Conclusively, truncating mutations within the P0 intracellular domain do not necessarily cause a severe phenotype such as Dejerine-Sottas syndrome (DSS) or congenital hypomyelinating neuropathy (CHN), but can result in mild or moderate CMT1B with intrafamilial clinical variability. Copyright © 2001 Elsevier Science B.V. [less ▲]

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See detailPrésentation d’un nouveau modèle de relations spatiales et implémentation dans la structure 3D SSM
Billen, Roland ULg

Scientific conference (2001, November 15)

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See detailNaturalisme et pragmatisme
Leclercq, Bruno ULg

Conference (2001, November 13)

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See detailLes associations dans le système européen des comptes nationaux
Mertens de Wilmars, Sybille ULg

Conference given outside the academic context (2001)

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See detailUsing the backdoor. Undocumented Migrants in the EU
Martiniello, Marco ULg

Scientific conference (2001, November 12)

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See detailRecent progress in ring-opening polymerization
Lecomte, Philippe ULg

Scientific conference (2001, November 12)

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See detailOptimiser l'expression orale en travaillant sur les stratégies de communication (1re partie)
Dahmen, Manfred; Doppagne, Véronique ULg; Simons, Germain ULg

Conference given outside the academic context (2001)

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See detailReprésentations et Pratiques Alimentaires en Adolescence
D'Amore, Salvatore ULg

Conference (2001, November 11)

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See detailMyoglobinurie atypique: une série de cas en Belgique
Delguste, Catherine ULg; Cassart, Dominique ULg

Conference (2001, November 10)

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See detailLa prise en charge des troubles de la mémoire chez les traumatisés crâniens
Adam, Stéphane ULg

Scientific conference (2001, November 10)

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See detailIKKα and IKKβ have different roles in LTβR-mediated NF-κB activation.
Dejardin, Emmanuel ULg; Haas, Elvira; Ware, Carl et al

Conference (2001, November 09)

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See detailL'évaluation des troubles de l'attention chez les traumatisés crâniens
Adam, Stéphane ULg

Scientific conference (2001, November 09)

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See detailLa valorisation de la betterave fourragère dans l'alimentation de la vache laitière et du bétail à l'engraissement
Beckers, Yves ULg

Conference given outside the academic context (2001)

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See detailThe Condition of Felicity of a Blind Instrument
Melard, François ULg

Conference (2001, November 03)

This paper deals with an historical and ethnographic case : the operation of field laboratories in the sugar industry and their use of an instrument to measure a vegetable - the sugar beet. An optical ... [more ▼]

This paper deals with an historical and ethnographic case : the operation of field laboratories in the sugar industry and their use of an instrument to measure a vegetable - the sugar beet. An optical instrument – the polariscope - gives voice to sugar beets in order to settle agreements on the vegetable's industrial definition and financial value. The polariscope gained its strange authority through a historical and technical capacity for mediation : a mediation between sugar beet growers and sugar manufacturers (and their social interests), but especially a mediation between these social actors and the vegetable itself. I focus on the idea that, in fact, this instrument is “blind” and describe the sociological “exchanges of properties” that take place between human and non-human actors in an industrial, public, and low-tech setting. My study shows that this is a challenging and alternative way - far from scientific laboratories - by which knowledge and coordination are produced. [less ▲]

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See detailH[SUB]2[/SUB] temperature and self-absorption: analysis of Jovian auroral spectra obtained with the FUSE satellite
Gustin, Jacques ULg; Feldman, P. D.; Gérard, Jean-Claude ULg et al

in Bulletin of the American Astronomical Society (2001, November 01)

High-resolution spectra of the Jovian aurora have been obtained with unprecedented spectral resolution in the 900-1190 Ì· window with the the Far Ultraviolet Spectroscopic Explorer (FUSE), using the 30 ... [more ▼]

High-resolution spectra of the Jovian aurora have been obtained with unprecedented spectral resolution in the 900-1190 Ì· window with the the Far Ultraviolet Spectroscopic Explorer (FUSE), using the 30"x30" LWRS aperture. All observed features belong to the H[SUB]2[/SUB] transitions from the B, C, B', D, B" and D' electronic states to the ground-state. These emissions are excited by inelastic collisions of the primary and secondary auroral electrons with H[SUB]2[/SUB] molecules. The relative intensity distribution of the observed lines depends on the rotational temperature of the emitting layer and self-absorption. Below 1100 Ì· , the transitions leading to the v" = 0, 1 and 2 levels of ground-state are partially or totally absorbed by H[SUB]2[/SUB], giving indications about the vibrational H[SUB]2[/SUB] distribution and overlying column. After a validation with an unabsorbed and a self-absorbed laboratory spectrum obtained in controlled conditions (100K, 300 eV), this study compares the observations and synthetic spectra, generated by a code including the B, C and B', D, B" and D' Rydberg states. The rotational and vibrational H[SUB]2[/SUB] temperatures are determined as well as the overlying H[SUB]2[/SUB] column. The combination of these parameters is used to determine the depth of the auroral energy deposition. This work is based on data obtained for the Guaranteed Time Team by the NASA-CNES-CSA FUSE mission operated by the Johns Hopkins University. French participants are supported by CNES. Financial support to U.S. participants has been provided by NASA contract NAS5-32985. [less ▲]

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See detailThe influence of gas depth on interfacial convection in a liquid gas bilayer.
Narayanan, Ranga; Theisen, Eric; Johnson, Duane et al

in Bulletin of the American Physical Society, Proceedings of the 54th Annual Meeting of the APS Division of Fluid Dynamics (2001, November 01)

We present a study of the influence of air height on the pattern formation at the onset of interfacial convection in a liquid -gas bilayer. Bilayer convection results from interaction of two immiscible ... [more ▼]

We present a study of the influence of air height on the pattern formation at the onset of interfacial convection in a liquid -gas bilayer. Bilayer convection results from interaction of two immiscible, superposed fluid layers and it is a transport problem that is affected by the ratios of various thermophysical properties and geometries. By using both calculations and experiments, a new convection-coupling mechanism is revealed. When the gas depth is low the convection is dominant in the liquid. It is of the Rayleigh Marangoni type and subcritical convection may be seen for axisymmetric patterns at the onset. For large gas heights the convection in the upper fluid layer simultaneously generates transverse thermal gradients along the fluid-fluid interface immediately causing surface tension gradient and buoyancy driven-convection in the lower fluid. The convection in the gas is of the Rayleigh type and the self-adjoint nature of the mathematical problem is depicted in the experiment wherein no subcritical motion occurs. In both the calculations and the experiment, silicone oil and air were used as the two fluids. As the viscosity of air is quite low compared to the silicone oil, convection in the air has no mechanical influence on the silicone oil and it is this property that is advantageous in isolating the new convection mechanism. The correspondence between theory and experiment is excellent. [less ▲]

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See detailAnorthosites: the lower crustal connection
Duchesne, Jean-Clair ULg

Conference (2001, November 01)

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See detailThe European Large Area ISO Survey - VI. Discovery of a new hyperluminous infrared galaxy
Morel, Thierry ULg; Efstathiou, A.; Serjeant, S. et al

in Monthly Notices of the Royal Astronomical Society (2001), 327

We report the discovery of the first hyperluminous infrared galaxy (HyLIG) in the course of the European Large Area ISO Survey (ELAIS). This object has been detected by ISO at 6.7, 15 and 90μm, and is ... [more ▼]

We report the discovery of the first hyperluminous infrared galaxy (HyLIG) in the course of the European Large Area ISO Survey (ELAIS). This object has been detected by ISO at 6.7, 15 and 90μm, and is found to be a broad-line, radio-quiet quasar at a redshift [formmu4]z=1.099. From a detailed multicomponent model fit of the spectral energy distribution, we derive a total IR luminosity L[SUB]IR[/SUB] [formmu5](1-1000μm)~1.0à 10[SUP]13[/SUP]h65-2L[SUB]solar[/SUB] [formmu6](q[SUB]0[/SUB]=0.5), and discuss the possible existence of a starburst contributing to the far-IR output. Observations to date present no evidence for lens magnification. This galaxy is one of the very few HyLIGs with a X-ray detection. On the basis of its soft X-ray properties, we suggest that this broad-line object may be the face-on analogue of narrow-line, Seyfert-like HyLIGs. [less ▲]

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See detailSpatial and temporal variations of the Jovian auroral electrons deduced from HST-STIS ultraviolet spectroscopy
Gérard, Jean-Claude ULg; Gustin, Jacques ULg; Grodent, Denis ULg et al

in Bulletin of the American Astronomical Society (2001, November 01)

Jovian auroral spectra have been observed with the Space Telescope Imaging Spectrometer (STIS) in both hemispheres since 1997, including during the recent Cassini Jupiter millenium campaign. The time ... [more ▼]

Jovian auroral spectra have been observed with the Space Telescope Imaging Spectrometer (STIS) in both hemispheres since 1997, including during the recent Cassini Jupiter millenium campaign. The time-tagged G140L STIS spectra used for this study cover the 1100-1700 Ì· range which includes the Lyman (B-X) bands and continuum and the Werner (C-X) bands. Time-dependent spectroscopy with spatial resolution along the 25 arcsec slit makes it possible to follow spectral and temporal variations in different regions of the aurora. FUV images observed on the same HST orbit were obtained to put the spectral data into the global morphology context. Synchronous observations of spectral domains absorbed or not by CH4 and other hydrocarbons are used to determine the FUV color ratio and the depth of the auroral emission relative to the methane homopause. Large variations of the color ratio across the auroral oval are observed. The time evolution of the mean electron energy and its possible correlation with the precipitated energy flux are investigated. It is found that the mean electron energy generally positively correlates with the auroral brightness. In contrast, flares occasionally observed in the polar cap can correspond to a softening or a hardening of the precipitation. The mean electron energy associated with the Io trail is approximately constant and shows less hydrocarbon absorption than the higher latitude aurora. [less ▲]

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See detailHST Observations of Aurora from the Magnetic Footprints of Io, Ganymede, and Europa during the Millennium Campaign
Clarke, J. T.; Grodent, Denis ULg; Connerney, J. et al

in Bulletin of the American Astronomical Society (2001, November 01)

UV images of Jupiter's aurora obtained with the HST STIS instrument reveal much detail in the distribution of emissions from Io's magnetic footprint on Jupiter, including extended trails of emission in ... [more ▼]

UV images of Jupiter's aurora obtained with the HST STIS instrument reveal much detail in the distribution of emissions from Io's magnetic footprint on Jupiter, including extended trails of emission in the downstream direction. Emissions are also seen from the magnetic footprints of Ganymede and Europa, relatively much fainter and point-like in spatial extent. Knowledge of the statistical properties of these emissions, in terms of their locations and brightnesses, was greatly advanced in a comprehensive series of images obtained during the Millennium campaign in Dec. 2000 - Jan. 2001. These images provide sufficient spatial coverage to give a good indication of the auroral oval locations mapping to Io and Ganymede, and a few points mapping to Europa. The observed variations in footprint properties provide indications of the nature of the electrodynamic interactions of the satellites with Jupiter's magnetic field. These and other indicators also show the direction that magnetic field models should take to better describe Jupiter's internal magnetic field. Contributing evidence comes from the locus of footprint latitudes, and their deviations from the VIP4 model. The latitudinal distance between the Io and Ganymede footprint loci indicates variations in the local field strength, with larger separations corresponding to a weaker field. These separations, and the distance from and distortion of the main oval, indicate an anomalously weak field region in the north near 90[SUP]o[/SUP] longitude. This work has been supported by NASA in STScI grants GO-08171-97A and GO-08657-01A to the University of Michigan. [less ▲]

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