457 resultados para Huntington
Resumo:
Huntington's disease is a polyglutamine expansion disorder, characterized by mutant HTT-mediated aggregate formation and cytotoxicity. Many reports suggests roles of N-terminal 17 amino acid domain of HTT (HTT-N17) towards subcellular localization, aggregate formation and subsequent pathogenicity induced by N-terminal HTT harboring polyQ stretch in pathogenic range. HYPK is a HTT-interacting chaperone which can reduce N-terminal mutant HTT-mediated aggregate formation and cytotoxicity in neuronal cell lines. However, how HYPK interacts with N-terminal fragment of HTT remained unknown. Here we report that specific interaction of HYPK with HTT-N17 is crucial for the chaperone activity of HYPK. Deletion of HTT-N17 leads to formation of tinier, SDS-soluble nuclear aggregates formed by N-terminal mutant HTT. The increased cytotoxicity imparted by these tiny aggregates might be contributed due to loss of interaction with HYPK. (C) 2014 Elsevier Inc. All rights reserved.
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Polydora nuchalis Woodwick, 1953 (Polychaeta: Spionidae) is a protandric hermaphrodite commonly inhabiting intertidal mud flats in southern California. The species exhibits lecithotrophic larval development and adelphophagia. Reproduction of P. nuchalis was monitored for a year at four sites: Catalina Harbor, San Gabriel River, Huntington Harbour, and Malibu Lagoon. Females deposited from 11 to 31 egg capsules in their tubes, with up to 230 eggs per capsule. An average of 3% of the eggs developed into larvae: the remaining were nurse eggs serving as food for the developing larvae. Reproductive output was quantified by determining the number and size of larvae and nurse eggs for individual capsules. Significant differences among the four populations were found for all the quantified variables. In addition, two size classes of nurse eggs were found to exist in capsules from all of the sites. Egg capsules were found throughout the year at San Gabriel River, but none were found during the winter months at the remaining three sites. Size/frequency data for juveniles and adults of the Catalina Harbor population indicate an annual cycle of recruitment. The laboratory experiment consisted of a 3 x 3 x 2 £actor1al design with replication testing the effects of temperature, salinity, and food supply on growth and reproduction of P. nuchalis. Increasing temperature resulted in significantly increased survivorship, growth rates, and percentage reproduction. It also produced a significant decrease in the size of the nurse eggs and the volume of food per larva. The number of egg capsules was maximum at the intermediate temperature. Increasing the salinity resulted in significant increases in survivorship and Class I nurse egg size. Increaaing food availability produced a significant increase in the percentage of worms reproducing. The interactive effect of salinity and £ood level produced significant changes in the number of larvae per capsule and the number of nurse eggs per capsule. However, the number of nurse eggs per larva did not differ significantly among the experimental treatment groups. (PDF contains 129 pages)
Resumo:
Hydrogen is the only atom for which the Schr odinger equation is solvable. Consisting only of a proton and an electron, hydrogen is the lightest element and, nevertheless, is far from being simple. Under ambient conditions, it forms diatomic molecules H2 in gas phase, but di erent temperature and pressures lead to a complex phase diagram, which is not completely known yet. Solid hydrogen was rst documented in 1899 [1] and was found to be isolating. At higher pressures, however, hydrogen can be metallized. In 1935 Wigner and Huntington predicted that the metallization pressure would be 25 GPa [2], where molecules would disociate to form a monoatomic metal, as alkali metals that lie below hydrogen in the periodic table. The prediction of the metallization pressure turned out to be wrong: metallic hydrogen has not been found yet, even under a pressure as high as 320 GPa. Nevertheless, extrapolations based on optical measurements suggest that a metallic phase may be attained at 450 GPa [3]. The interest of material scientist in metallic hydrogen can be attributed, at least to a great extent, to Ashcroft, who in 1968 suggested that such a system could be a hightemperature superconductor [4]. The temperature at which this material would exhibit a transition from a superconducting to a non-superconducting state (Tc) was estimated to be around room temperature. The implications of such a statement are very interesting in the eld of astrophysics: in planets that contain a big quantity of hydrogen and whose temperature is below Tc, superconducting hydrogen may be found, specially at the center, where the gravitational pressure is high. This might be the case of Jupiter, whose proportion of hydrogen is about 90%. There are also speculations suggesting that the high magnetic eld of Jupiter is due to persistent currents related to the superconducting phase [5]. Metallization and superconductivity of hydrogen has puzzled scientists for decades, and the community is trying to answer several questions. For instance, what is the structure of hydrogen at very high pressures? Or a more general one: what is the maximum Tc a phonon-mediated superconductor can have [6]? A great experimental e ort has been carried out pursuing metallic hydrogen and trying to answer the questions above; however, the characterization of solid phases of hydrogen is a hard task. Achieving the high pressures needed to get the sought phases requires advanced technologies. Diamond anvil cells (DAC) are commonly used devices. These devices consist of two diamonds with a tip of small area; for this reason, when a force is applied, the pressure exerted is very big. This pressure is uniaxial, but it can be turned into hydrostatic pressure using transmitting media. Nowadays, this method makes it possible to reach pressures higher than 300 GPa, but even at this pressure hydrogen does not show metallic properties. A recently developed technique that is an improvement of DAC can reach pressures as high as 600 GPa [7], so it is a promising step forward in high pressure physics. Another drawback is that the electronic density of the structures is so low that X-ray di raction patterns have low resolution. For these reasons, ab initio studies are an important source of knowledge in this eld, within their limitations. When treating hydrogen, there are many subtleties in the calculations: as the atoms are so light, the ions forming the crystalline lattice have signi cant displacements even when temperatures are very low, and even at T=0 K, due to Heisenberg's uncertainty principle. Thus, the energy corresponding to this zero-point (ZP) motion is signi cant and has to be included in an accurate determination of the most stable phase. This has been done including ZP vibrational energies within the harmonic approximation for a range of pressures and at T=0 K, giving rise to a series of structures that are stable in their respective pressure ranges [8]. Very recently, a treatment of the phases of hydrogen that includes anharmonicity in ZP energies has suggested that relative stability of the phases may change with respect to the calculations within the harmonic approximation [9]. Many of the proposed structures for solid hydrogen have been investigated. Particularly, the Cmca-4 structure, which was found to be the stable one from 385-490 GPa [8], is metallic. Calculations for this structure, within the harmonic approximation for the ionic motion, predict a Tc up to 242 K at 450 GPa [10]. Nonetheless, due to the big ionic displacements, the harmonic approximation may not su ce to describe correctly the system. The aim of this work is to apply a recently developed method to treat anharmonicity, the stochastic self-consistent harmonic approximation (SSCHA) [11], to Cmca-4 metallic hydrogen. This way, we will be able to study the e ects of anharmonicity in the phonon spectrum and to try to understand the changes it may provoque in the value of Tc. The work is structured as follows. First we present the theoretical basis of the calculations: Density Functional Theory (DFT) for the electronic calculations, phonons in the harmonic approximation and the SSCHA. Then we apply these methods to Cmca-4 hydrogen and we discuss the results obtained. In the last chapter we draw some conclusions and propose possible future work.
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The population of belugas, Delphinapterus leucas, in Cook Inlet, Alaska, is geographically isolated and appears to be declining. Conservation efforts require appropriate information about population levels and trends, feeding and behavior, reproduction, and natural and anthropogenic impacts. This study documents traditional ecological knowledge of the Alaska Native hunters of belugas in Cook Inlet to add information from this critical source. Traditional knowledge about belugas has been documented elsewhere by the author, and the same methods were used in Cook Inlet to systematically gather information concerning knowledge of the natural history of this beluga population and its habitat. The hunters’knowledge is largely consistent with what is known from previous research, and it extends the published descriptions of the ecology of beluga whales in Cook Inlet. Making this information available and involving the hunters to a greater extent in research and management are important contributions to the conservation of Cook Inlet beluga
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The electronic band structures and optical gains of InAs1-xNx/GaAs pyramid quantum dots (QDs) are calculated using the ten-band k . p model and the valence force field method. The optical gains are calculated using the zero-dimensional optical gain formula with taking into consideration of both homogeneous and inhomogeneous broadenings due to the size fluctuation of quantum dots which follows a normal distribution. With the variation of QD sizes and nitrogen composition, it can be shown that the nitrogen composition and the strains can significantly affect the energy levels especially the conduction band which has repulsion interaction with nitrogen resonant state due to the band anticrossing interaction. It facilitates to achieve emission of longer wavelength (1.33 or 1.55 mu m) lasers for optical fiber communication system. For QD with higher nitrogen composition, it has longer emission wavelength and less detrimental effect of higher excited state transition, but nitrogen composition can affect the maximum gain depending on the factors of transition matrix element and the Fermi-Dirac distributions for electrons in the conduction bands and holes in the valence bands respectively. For larger QD, its maximum optical gain is greater at lower carrier density, but it is slowly surpassed by smaller QD as carrier concentration increases. Larger QD can reach its saturation gain faster, but this saturation gain is smaller than that of smaller QD. So the trade-off between longer wavelength, maximum optical, saturation gain, and differential gain must be considered to select the appropriate QD size according to the specific application requirement. (C) 2009 American Institute of Physics. [DOI: 10.1063/1.3143025]
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Tunneling magnetoresistance (TMR) in Ga(0.9)2Mn(0.08)As/Al-O/Co40Fe40B20 trilayer hybrid structure as a function of temperature from 10 to 50 K with magnetic field vertical bar H vertical bar <= 2000 Oe has been studied. TMR ratio of 1.6% at low fields at 10 K was achieved with the applied current of 1 mu A. The behavior of junction resistance was well explained by the tunneling resistance across the barrier. Strong bias dependences of magnetoresistance and junction resistance were presented. (C) 2009 American Institute of Physics. [DOI: 10.1063/1.3068418]
Resumo:
In this paper we consider the continuous weak measurement of a solid-state qubit by single electron transistors (SET). For single-dot SET, we find that in nonlinear response regime the signal-to-noise ratio can violate the universal upper bound imposed quantum mechanically on any linear response detectors. We understand the violation by means of the cross-correlation of the detector currents. For double-dot SET, we discuss its robustness against wider range of temperatures, quantum efficiency, and the relevant open issues unresolved.
Resumo:
The effect of thermal annealing on the Raman spectrum of Si0.33Ge0.67 alloy grown on Si (100) by molecular beam epitaxy is investigated in the temperature range of 550-800 degrees C. For annealing below 700 degrees C, interdiffusion at the interface is negligible and the residual strain plays the dominant role in the Raman shift. The strain-shift coefficients for Si-Ge and Ge-Ge phonon modes are determined to be 915 +/- 215 cm(-1) and 732 +/- 117 cm(-1), respectively. For higher temperature annealing, interdiffusion is significant and strongly affects the Raman shift and the spectral shape.
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A semi-insulating (SI) GaAs single crystal ingot was successfully grown in a recoverable satellite. The two-dimensional distribution of stoichiometry in space-grown SI-GaAs single crystal wafer was studied nondestructively based upon x-ray Band diffraction. The avenge stoichiometry in the space-grown crystal is 0.50007 with mean square deviation of 6 x 10(-6), and shows a better stoichiametric property than the ground-grown SI-GaAs. The average etch pit density (EPD) of dislocations in the crystal revealed by molten KOH is 2.0 x 10(4) cm(-2), and the highest EPD is 3.1 x 10(4) cm(-2). This result indicates that the structural properly of the crystal is quite good.
Resumo:
We show that part of the reflectance difference resonance near the E-0 energy of ZnSe is due to the anisotropic in-plane strain in the ZnSe thin films, as films grown on three distinctly different substrates, GaAs, GaP, and ZnS, all show the resonance at the same energy. Such anisotropic strain induced resonance is predicted and also observed near the E-1/E-1+Delta(1) energies in ZnSe grown on GaAs. The theory also predicts that there should be no resonance due to strain at, the E-0+Delta(0) energy, which is consistent with experiments. The strain anisotropy is rather independent of the ZnSe layer thickness, or whether the film is strain relaxed. For ZnSe films with large lattice mismatch with substrates, the resonance at the E-1/E-1+Delta(1) energies is absent, very likely due to the poor crystalline quality of the 20 nm or so surface layer. (C) 2000 American Vacuum Society. [S0734-211X(00)05604-3].
Resumo:
Prescott, Sarah, ''What Foes more dang'rous than too strong Allies?': Anglo-Welsh relations in eighteenth-century London', The Huntington Library Quarterly (2006) 69 (4) pp.535-554 RAE2008
Resumo:
Co-release of the inhibitory neurotransmitter GABA and the neuropeptide substance-P (SP) from single axons is a conspicuous feature of the basal ganglia, yet its computational role, if any, has not been resolved. In a new learning model, co-release of GABA and SP from axons of striatal projection neurons emerges as a highly efficient way to compute the uncertainty responses that are exhibited by dopamine (DA) neurons when animals adapt to probabilistic contingencies between rewards and the stimuli that predict their delivery. Such uncertainty-related dopamine release appears to be an adaptive phenotype, because it promotes behavioral switching at opportune times. Understanding the computational linkages between SP and DA in the basal ganglia is important, because Huntington's disease is characterized by massive SP depletion, whereas Parkinson's disease is characterized by massive DA depletion.
Resumo:
This study contexualises the relationship between the armed forces and the civil authority in Ireland using and revising the theoretical framework advanced by Huntington. It tracks the evolution of the idea of a representive body for soldiers in the late 1980s, to the setting up of statutory associations under the Defence Amendment Act 1990. The study considers Irish soldiers political agitation and their use of peaceful democratic activities to achieve their aims. It highlights the fundamental policy arguments that were made against the idea of representation for the army and positions those arguments in the study of civil-military relations. Utilising unique access to secret Department of Defence files, it reveals in-depth ideological arguments advanced by the military authories in Ireland against independent representation. This thesis provides an academic study of the establishment of PDFORRA. It answers key questions regarding the change in the position of Irish government who were categorically opposed to the idea of representation in the army. It illustrates the involvement of other agencies such as the European Organisation of Military Associations (Euromil) reveals reciprocal support by the Irish associations to other emerging groups in Spain. Accessing as yet unpublished Department of Defence files, study analyses tension between the military authorities and the government. It highlights for the first time the role of enlisted personnel in the shaping of new state structures and successfully dismmisses Huntingtons theoretical contention that enlisted personnel are of no consequence in the study of civil-military relations. It fills a gap in our understanding, identified by Finer, as to how politicisation of soldiers takes place. This thesis brings a new dimension to the discipline of civil-military relations and creates new knowledge that will enhance our understanding of an area not covered previously.