973 resultados para Ataxie spinocérébelleuse de type-3 (SCA3)


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The structure and properties of the diffuse interstellar medium (ISM) on small scales, sub-au to 1 pc, are poorly understood. We compare interstellar absorption-lines, observed towards a selection of O- and B-type stars at two or more epochs, to search for variations over time caused by the transverse motion of each star combined with changes in the structure in the foreground ISM. Two sets of data were used: 83 VLT- UVES spectra with approximately 6 yr between epochs and 21 McDonald observatory 2.7m telescope echelle spectra with 6 - 20 yr between epochs, over a range of scales from 0 - 360 au. The interstellar absorption-lines observed at the two epochs were subtracted and searched for any residuals due to changes in the foreground ISM. Of the 104 sightlines investigated with typically five or more components in Na I D, possible temporal variation was identified in five UVES spectra (six components), in Ca II, Ca I and/or Na I absorption-lines. The variations detected range from 7\% to a factor of 3.6 in column density. No variation was found in any other interstellar species. Most sightlines show no variation, with 3{\sigma} upper limits to changes of the order 0.1 - 0.3 dex in Ca II and Na I. These variations observed imply that fine-scale structure is present in the ISM, but at the resolution available in this study, is not very common at visible wavelengths. A determination of the electron densities and lower limits to the total number density of a sample of the sightlines implies that there is no striking difference between these parameters in sightlines with, and sightlines without, varying components.

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We present optical and near-infrared photometry and spectroscopy of SN 2009ib, a Type II-P supernova in NGC 1559. This object has moderate brightness, similar to those of the intermediate-luminosity SNe 2008in and 2009N. Its plateau phase is unusually long, lasting for about 130 d after explosion. The spectra are similar to those of the subluminous SN 2002gd, with moderate expansion velocities. We estimate the Ni-56 mass produced as 0.046 +/- A 0.015 M-aS (TM). We determine the distance to SN 2009ib using both the expanding photosphere method (EPM) and the standard candle method. We also apply EPM to SN 1986L, a Type II-P SN that exploded in the same galaxy. Combining the results of different methods, we conclude the distance to NGC 1559 as D = 19.8 +/- A 3.0 Mpc. We examine archival, pre-explosion images of the field taken with the Hubble Space Telescope, and find a faint source at the position of the SN, which has a yellow colour [(V - I)(0) = 0.85 mag]. Assuming it is a single star, we estimate its initial mass as M-ZAMS = 20 M-aS (TM). We also examine the possibility, that instead of the yellow source the progenitor of SN 2009ib is a red supergiant star too faint to be detected. In this case, we estimate the upper limit for the initial zero-age main sequence (ZAMS) mass of the progenitor to be similar to 14-17 M-aS (TM). In addition, we infer the physical properties of the progenitor at the explosion via hydrodynamical modelling of the observables, and estimate the total energy as similar to 0.55 x 10(51) erg, the pre-explosion radius as similar to 400 R-aS (TM), and the ejected envelope mass as similar to 15 M-aS (TM), which implies that the mass of the progenitor before explosion was similar to 16.5-17 M-aS (TM).

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Stellar evolution models predict the existence of hybrid white dwarfs (WDs) with a carbon-oxygen core surrounded by an oxygen-neon mantle. Being born with masses similar to 1.1 M-aS (TM), hybrid WDs in a binary system may easily approach the Chandrasekhar mass (M-Ch) by accretion and give rise to a thermonuclear explosion. Here, we investigate an off-centre deflagration in a near-M-Ch hybrid WD under the assumption that nuclear burning only occurs in carbon-rich material. Performing hydrodynamics simulations of the explosion and detailed nucleosynthesis post-processing calculations, we find that only 0.014 M-aS (TM) of material is ejected while the remainder of the mass stays bound. The ejecta consist predominantly of iron-group elements, O, C, Si and S. We also calculate synthetic observables for our model and find reasonable agreement with the faint Type Iax SN 2008ha. This shows for the first time that deflagrations in near-M-Ch WDs can in principle explain the observed diversity of Type Iax supernovae. Leaving behind a near-M-Ch bound remnant opens the possibility for recurrent explosions or a subsequent accretion-induced collapse in faint Type Iax SNe, if further accretion episodes occur. From binary population synthesis calculations, we find the rate of hybrid WDs approaching M-Ch to be of the order of 1 per cent of the Galactic SN Ia rate.

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We present the GALEX detection of a UV burst at the time of explosion of an optically normal supernova (SN) IIP (PS1-13arp) from the Pan-STARRS1 survey at z = 0.1665. The temperature and luminosity of the UV burst match the theoretical predictions for shock breakout in a red supergiant (RSG), but with a duration a factor of similar to 50 longer than expected. We compare the NUV light curve of PS1-13arp to previous GALEX detections of SNe IIP and find clear distinctions that indicate that the UV emission is powered by shock breakout, and not by the subsequent cooling envelope emission previously detected in these systems. We interpret the similar to 1 day duration of the UV signal with a shock breakout in the wind of an RSG with a pre-explosion mass-loss rate of similar to 10(-3) M-circle dot yr(-1). This mass-loss rate is enough to prolong the duration of the shock breakout signal, but not enough to produce an excess in the optical plateau light curve or narrow emission lines powered by circumstellar interaction. This detection of nonstandard, potentially episodic high mass loss in an RSG SN progenitor has favorable consequences for the prospects of future wide-field UV surveys to detect shock breakout directly in these systems, and provide a sensitive probe of the pre-explosion conditions of SN progenitors.

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We investigate line formation processes in Type IIb supernovae (SNe) from 100 to 500 days post-explosion using spectral synthesis calculations. The modelling identifies the nuclear burning layers and physical mechanisms that produce the major emission lines, and the diagnostic potential of these. We compare the model calculations with data on the three best observed Type IIb SNe to-date - SN 1993J, SN 2008ax, and SN 2011dh. Oxygen nucleosynthesis depends sensitively on the main-sequence mass of the star and modelling of the [O I] lambda lambda 6300, 6364 lines constrains the progenitors of these three SNe to the M-ZAMS = 12-16 M-circle dot range (ejected oxygen masses 0.3-0.9 M-circle dot), with SN 2011dh towards the lower end and SN 1993J towards the upper end of the range. The high ejecta masses from M-ZAMS greater than or similar to 17 M-circle dot progenitors give rise to brighter nebular phase emission lines than observed. Nucleosynthesis analysis thus supports a scenario of low-to-moderate mass progenitors for Type IIb SNe, and by implication an origin in binary systems. We demonstrate how oxygen and magnesium recombination lines may be combined to diagnose the magnesium mass in the SN ejecta. For SN 2011dh, a magnesium mass of 0.02-0.14 M-circle dot is derived, which gives a Mg/O production ratio consistent with the solar value. Nitrogen left in the He envelope from CNO burning gives strong [N II] lambda lambda 6548, 6583 emission lines that dominate over Ha emission in our models. The hydrogen envelopes of Type IIb SNe are too small and dilute to produce any noticeable H alpha emission or absorption after similar to 150 days, and nebular phase emission seen around 6550 angstrom is in many cases likely caused by [N II] lambda lambda 6548, 6583. Finally, the influence of radiative transport on the emergent line profiles is investigated. Significant line blocking in the metal core remains for several hundred days, which affects the emergent spectrum. These radiative transfer effects lead to early-time blueshifts of the emission line peaks, which gradually disappear as the optical depths decrease with time. The modelled evolution of this effect matches the observed evolution in SN 2011dh.

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Purpose: Although L-type Ca2+ channels are known to play a key role in the myogenic reactivity of retinal arterial vessels, the involvement of other types of voltage-gated Ca2+ channels in this process remains unknown. In the present study we have investigated the contribution of T-type Ca2+ channels to myogenic signalling in arterioles of the rat retinal microcirculation.

Methods: Confocal immunolabelling of wholemount preparations was used to investigate the localisation of CaV3.1-3 channels in retinal arteriolar smooth muscle cells. T-type currents and the contribution of T-type channels to myogenic signalling were assessed by whole-cell patch-clamp recording and pressure myography of isolated retinal arteriole segments.

Results: Strong immunolabelling for CaV3.1 was observed on the plasma membrane of retinal arteriolar smooth muscle cells. In contrast, no expression of CaV3.2 or CaV3.3 could be detected in retinal arterioles, although these channels were present on glial cell end feet surrounding the vessels and retinal ganglion cells, respectively. TTA-A2 sensitive T-type currents were recorded in retinal arteriolar myocytes with biophysical properties distinct from those of the L-type currents present in these cells. Inhibition of T-type channels using TTA-A2 or ML-218 dilated isolated, myogenically active, retinal arterioles.

Conclusions: CaV3.1 T-type Ca2+ channels are functionally expressed on arteriolar smooth muscle cells of retinal arterioles and play an important role in myogenic signalling in these vessels. The work has important implications concerning our understanding of the mechanisms controlling blood flow autoregulation in the retina and its disruption during ocular disease.

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We probe the systematic uncertainties from the 113 Type Ia supernovae (SN Ia) in the Pan-STARRS1 (PS1) sample along with 197 SN Ia from a combination of low-redshift surveys. The companion paper by Rest et al. describes the photometric measurements and cosmological inferences from the PS1 sample. The largest systematic uncertainty stems from the photometric calibration of the PS1 and low-z samples. We increase the sample of observed Calspec standards from 7 to 10 used to define the PS1 calibration system. The PS1 and SDSS-II calibration systems are compared and discrepancies up to ∼0.02 mag are recovered. We find uncertainties in the proper way to treat intrinsic colors and reddening produce differences in the recovered value of w up to 3%. We estimate masses of host galaxies of PS1 supernovae and detect an insignificant difference in distance residuals of the full sample of 0.037 ± 0.031 mag for host galaxies with high and low masses. Assuming flatness and including systematic uncertainties in our analysis of only SNe measurements, we find w = -1.120+0.360-0.206(Stat)+0.269-0.291(Sys). With additional constraints from Baryon acoustic oscillation, cosmic microwave background (CMB) (Planck) and H0 measurements, we find w = -1.166+0.072-0.069 and Ωm = 0.280+0.013-0.012 (statistical and systematic errors added in quadrature). The significance of the inconsistency with w = -1 depends on whether we use Planck or Wilkinson Microwave Anisotropy Probe measurements of the CMB: wBAO+H0+SN+WMAP = -1.124+0.083-0.065.

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We present grizP1 light curves of 146 spectroscopically confirmed Type Ia supernovae (SNe Ia; 0.03 < z < 0.65) discovered during the first 1.5 yr of the Pan-STARRS1 Medium Deep Survey. The Pan-STARRS1 natural photometric system is determined by a combination of on-site measurements of the instrument response function and observations of spectrophotometric standard stars. We find that the systematic uncertainties in the photometric system are currently 1.2% without accounting for the uncertainty in the Hubble Space Telescope Calspec definition of the AB system. A Hubble diagram is constructed with a subset of 113 out of 146 SNe Ia that pass our light curve quality cuts. The cosmological fit to 310 SNe Ia (113 PS1 SNe Ia + 222 light curves from 197 low-z SNe Ia), using only supernovae (SNe) and assuming a constant dark energy equation of state and flatness, yields w = -1.120+0.360-0.206(Stat)+0.2690.291(Sys). When combined with BAO+CMB(Planck)+H0, the analysis yields ΩM = 0.280+0.0130.012 and w = -1.166+0.072-0.069 including all identified systematics. The value of w is inconsistent with the cosmological constant value of -1 at the 2.3σ level. Tension endures after removing either the baryon acoustic oscillation (BAO) or the H0 constraint, though it is strongest when including the H0 constraint. If we include WMAP9 cosmic microwave background (CMB) constraints instead of those from Planck, we find w = -1.124+0.083-0.065, which diminishes the discord to <2σ. We cannot conclude whether the tension with flat ΛCDM is a feature of dark energy, new physics, or a combination of chance and systematic errors. The full Pan-STARRS1 SN sample with ∼three times as many SNe should provide more conclusive results.

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We present an extensive optical and near-infrared photometric and spectroscopic campaign of the Type IIP supernova SN 2012aw. The data set densely covers the evolution of SN 2012aw shortly after the explosion through the end of the photospheric phase, with two additional photometric observations collected during the nebular phase, to fit the radioactive tail and estimate the 56Ni mass. Also included in our analysis is the previously published Swift UV data, therefore providing a complete view of the ultraviolet-optical- infrared evolution of the photospheric phase. On the basis of our data set, we estimate all the relevant physical parameters of SN 2012aw with our radiation-hydrodynamics code: envelope mass M env ∼ 20 M , progenitor radius R ∼ 3 × 1013 cm (∼430 R), explosion energy E ∼ 1.5 foe, and initial 56Ni mass ∼0.06 M. These mass and radius values are reasonably well supported by independent evolutionary models of the progenitor, and may suggest a progenitor mass higher than the observational limit of 16.5 ± 1.5 M of the Type IIP events. 

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We present optical and infrared monitoring data of SN 2012hn collectedby the Public European Southern Observatory Spectroscopic Survey forTransient Objects. We show that SN 2012hn has a faint peak magnitude(MR ˜ -15.65) and shows no hydrogen and no clearevidence for helium in its spectral evolution. Instead, we detectprominent Ca II lines at all epochs, which relates this transient topreviously described `Ca-rich' or `gap' transients. However, thephotospheric spectra (from -3 to +32 d with respect to peak) of SN2012hn show a series of absorption lines which are unique and a redcontinuum that is likely intrinsic rather than due to extinction. Linesof Ti II and Cr II are visible. This may be a temperature effect, whichcould also explain the red photospheric colour. A nebular spectrum at+150 d shows prominent Ca II, O I, C I and possibly Mg I lines whichappear similar in strength to those displayed by core-collapsesupernovae (SNe). To add to the puzzle, SN 2012hn is located at aprojected distance of 6 kpc from an E/S0 host and is not close to anyobvious star-forming region. Overall SN 2012hn resembles a group offaint H-poor SNe that have been discovered recently and for which aconvincing and consistent physical explanation is still missing. Theyall appear to explode preferentially in remote locations offset from amassive host galaxy with deep limits on any dwarf host galaxies,favouring old progenitor systems. SN 2012hn adds heterogeneity to thissample of objects. We discuss potential explosion channels includingHe-shell detonations and double detonations of white dwarfs as well aspeculiar core-collapse SNe.

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We report the results of our search for the progenitor candidate of SN 2013dk, a Type Ic supernova (SN) that exploded in the Antennae galaxy system. We compare pre-explosion Hubble Space Telescope (HST) archival images with SN images obtained using adaptive optics at the ESO Very Large Telescope. We isolate the SN position to within 3σ uncertainty radius of 0.02 arcsec and show that there is no detectable point source in any of the HST filter images within the error circle. We set an upper limit to the absolute magnitude of the progenitor to be MF555W ≳ -5.7, which does not allow Wolf-Rayet (WR) star progenitors to be ruled out. A bright source appears 0.17 arcsec away, which is either a single bright supergiant or compact cluster, given its absolute magnitude of MF555W = -9.02 ± 0.28 extended wings and complex environment. However, even if this is a cluster, the spatial displacement of SN 2013dk means that its membership is not assured. The strongest statement that we can make is that in the immediate environment of SN 2013dk (within 10 pc or so), we find no clear evidence of either a point source coincident with the SN or a young stellar cluster that could host a massive WR progenitor.

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We present the Pan-STARRS1 discovery of PS1-10afx, a unique hydrogen-deficient superluminous supernova (SLSN) at redshift z = 1.388. The light curve peaked at z P1 = 21.7 mag, making PS1-10afx comparable to the most luminous known SNe, with Mu = -22.3 mag. Our extensive optical and near-infrared observations indicate that the bolometric light curve of PS1-10afx rose on the unusually fast timescale of ~12 days to the extraordinary peak luminosity of 4.1 × 1044 erg s-1 (M bol = -22.8 mag) and subsequently faded rapidly. Equally important, the spectral energy distribution is unusually red for an SLSN, with a color temperature of ~6800 K near maximum light, in contrast to previous hydrogen-poor SLSNe, which are bright in the ultraviolet (UV). The spectra more closely resemble those of a normal SN Ic than any known SLSN, with a photospheric velocity of ~11, 000 km s-1 and evidence for line blanketing in the rest-frame UV. Despite the fast rise, these parameters imply a very large emitting radius (gsim 5 × 1015 cm). We demonstrate that no existing theoretical model can satisfactorily explain this combination of properties: (1) a nickel-powered light curve cannot match the combination of high peak luminosity with the fast timescale; (2) models powered by the spindown energy of a rapidly rotating magnetar predict significantly hotter and faster ejecta; and (3) models invoking shock breakout through a dense circumstellar medium cannot explain the observed spectra or color evolution. The host galaxy is well detected in pre-explosion imaging with a luminosity near L*, a star formation rate of ~15 M ⊙ yr-1, and is fairly massive (~2 × 1010 M ⊙), with a stellar population age of ~108 yr, also in contrast to the young dwarf hosts of known hydrogen-poor SLSNe. PS1-10afx is distinct from known examples of SLSNe in its spectra, colors, light-curve shape, and host galaxy properties, suggesting that it resulted from a different channel than other hydrogen-poor SLSNe.

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We report on our serendipitous pre-discovery detection and follow-up observations of the broad-lined Type Ic supernova (SN Ic) 2010ay at z = 0.067 imaged by the Pan-STARRS1 3π survey just ~4 days after explosion. The supernova (SN) had a peak luminosity, MR ≈ -20.2 mag, significantly more luminous than known GRB-SNe and one of the most luminous SNe Ib/c ever discovered. The absorption velocity of SN 2010ay is v Si ≈ 19 × 103 km s-1 at ~40 days after explosion, 2-5 times higher than other broad-lined SNe and similar to the GRB-SN 2010bh at comparable epochs. Moreover, the velocity declines ~2 times slower than other SNe Ic-BL and GRB-SNe. Assuming that the optical emission is powered by radioactive decay, the peak magnitude implies the synthesis of an unusually large mass of 56Ni, M Ni = 0.9 M ⊙. Applying scaling relations to the light curve, we estimate a total ejecta mass, M ej ≈ 4.7 M ⊙, and total kinetic energy, EK ≈ 11 × 1051 erg. The ratio of M Ni to M ej is ~2 times as large for SN 2010ay as typical GRB-SNe and may suggest an additional energy reservoir. The metallicity (log (O/H)PP04 + 12 = 8.19) of the explosion site within the host galaxy places SN 2010ay in the low-metallicity regime populated by GRB-SNe, and ~0.5(0.2) dex lower than that typically measured for the host environments of normal (broad-lined) SNe Ic. We constrain any gamma-ray emission with E γ ~ 1048 erg. We therefore rule out the association of a relativistic outflow like those that accompanied SN 1998bw and traditional long-duration gamma-ray bursts (GRBs), but we place less-stringent constraints on a weak afterglow like that seen from XRF 060218. If this SN did not harbor a GRB, these observations challenge the importance of progenitor metallicity for the production of relativistic ejecta and suggest that other parameters also play a key role.

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Members of a large pedigree of Irish origin presenting with early onset Type I autosomal dominant retinitis pigmentosa (ADRP) have been typed for D3S47 (C17), a polymorphic marker from the long arm of chromosome 3. Significant, tight linkage of ADRP to D3S47, with a lod score of 14.7 maximizing at 0.00 recombination, has been obtained, hence localizing the ADRP gene (RP1) segregating in this pedigree to 3q.

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BACKGROUND: Dietary cocoa is an important source of flavonoids and is associated with favorable cardiovascular disease effects, such as improvements in vascular function and lipid profiles, in nondiabetic adults. Type 2 diabetes (T2D) is associated with adverse effects on postprandial serum glucose, lipids, inflammation, and vascular function.

OBJECTIVE: We examined the hypothesis that cocoa reduces metabolic stress in obese T2D adults after a high-fat fast-food-style meal.

METHODS: Adults with T2D [n = 18; age (means ± SEs): 56 ± 3 y; BMI (in kg/m(2)): 35.3 ± 2.0; 14 women; 4 men) were randomly assigned to receive cocoa beverage (960 mg total polyphenols; 480 mg flavanols) or flavanol-free placebo (110 mg total polyphenols; <0.1 mg flavanols) with a high-fat fast-food-style breakfast [766 kcal, 50 g fat (59% energy)] in a crossover trial. After an overnight fast (10-12 h), participants consumed the breakfast with cocoa or placebo, and blood sample collection [glucose, insulin, lipids, and high-sensitivity C-reactive protein (hsCRP)] and vascular measurements were conducted at 0.5, 1, 2, 4, and 6 h postprandially on each study day. Insulin resistance was evaluated by homeostasis model assessment.

RESULTS: Over the 6-h study, and specifically at 1 and 4 h, cocoa increased HDL cholesterol vs. placebo (overall Δ: 1.5 ± 0.8 mg/dL; P ≤ 0.01) but had no effect on total and LDL cholesterol, triglycerides, glucose, and hsCRP. Cocoa increased serum insulin concentrations overall (Δ: 5.2 ± 3.2 mU/L; P < 0.05) and specifically at 4 h but had no overall effects on insulin resistance (except at 4 h, P < 0.05), systolic or diastolic blood pressure, or small artery elasticity. However, large artery elasticity was overall lower after cocoa vs. placebo (Δ: -1.6 ± 0.7 mL/mm Hg; P < 0.05), with the difference significant only at 2 h.

CONCLUSION: Acute cocoa supplementation showed no clear overall benefit in T2D patients after a high-fat fast-food-style meal challenge. Although HDL cholesterol and insulin remained higher throughout the 6-h postprandial period, an overall decrease in large artery elasticity was found after cocoa consumption. This trial was registered at clinicaltrials.gov as NCT01886989.