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1.

Background  

Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder linked to expanded CAG-triplet nucleotide repeats within the huntingtin gene. Intracellular huntingtin aggregates are present in neurons of distinct brain areas, among them regions of adult neurogenesis including the hippocampus and the subventricular zone/olfactory bulb system. Previously, reduced hippocampal neurogenesis has been detected in transgenic rodent models of HD. Therefore, we hypothesized that mutant huntingtin also affects newly generated neurons derived from the subventricular zone of adult R6/2 HD mice.  相似文献   

2.
ABSTRACT: BACKGROUND: Cell therapy is a potential therapeutic approach for several neurodegenetative disease, including Huntington Disease (HD). To evaluate the putative efficacy of cell therapy in HD, most studies have used excitotoxic animal models with only a few studies having been conducted in genetic animal models. Genetically modified animals should provide a more accurate representation of human HD, as they emulate the genetic basis of its etiology. RESULTS: In this study, we aimed to assess the therapeutic potential of a human striatal neural stem cell line (STROC05) implanted in the R6/2 transgenic mouse model of HD. As DARPP-32 GABAergic output neurons are predominately lost in HD, STROC05 cells were also predifferentiated using purmorphamine, a hedgehog agonist, to yield a greater number of DARPP-32 cells. A bilateral injection of 4.5x105 cells of either undifferentiated or predifferentiated DARPP-32 cells, however, did not affect outcome compared to a vehicle control injection. Both survival and neuronal differentiation remained poor with a mean of only 161 and 81 cells surviving in the undifferentiated and differentiated conditions respectively. Only a few cells expressed the neuronal marker beta-III-tubulin. CONCLUSIONS: Although the rapid brain atrophy and short life-span of the R6/2 model constitute adverse conditions to detect potentially delayed treatment effects, significant technical hurdles, such as poor cell survival and differentiation, were also sub-optimal. Further consideration of these aspects is therefore needed in more enduring transgenic HD models to provide a definite assessment of this cell line's therapeutic relevance. However, a combination of treatments is likely needed to affect outcome in transgenic models of HD.  相似文献   

3.

Background  

In Huntington's disease (HD), age at neurological onset is inversely correlated with the length of the CAG trinucleotide repeat mutation, but can be modified by genetic factors beyond the HD gene. Association of a relatively infrequent 16 TAA allele of a trinucleotide repeat polymorphism in the GRIK2 3'UTR with earlier than expected age at neurological onset has been suggested to reflect linkage disequilibrium with a functional polymorphism in GRIK2 or an adjacent gene.  相似文献   

4.
Variants based on the assumption of effective hydrodynamic radius being a constant are usually adopted to test the Stokes–Einstein (SE) relation. The rationality of the assumption is examined by performing molecular dynamics simulations with the truncated Lennard-Jones-like (TLJ) model, Kob–Andersen model and ortho-terphenyl (OTP) model. The results indicate the assumption is generally not established except for special case. The effective hydrodynamic radius is observed to increase with decreasing temperature for TLJ model but is decreased for Kob–Andersen and OTP model; and which is almost a constant for TLJ particle with enough rigidity. The variant of SE relation $D\sim T/\eta $ is invalid for the three models except for the TLJ particle with enough rigidity. We propose similar inconsistency may be also existed in other liquids and the assumption should be critically evaluated when adopted to test the SE relation.  相似文献   

5.
Electron spin resonance in a single-ground-state magnet, NiSnCl6·6H2O, has been studied at temperatures between 83 mK and 4.2 K. Below 1 K, the resonance lines show marked shifts, which can be described quantitatively in terms of exchange shift due to polarization effects treated by McMillan and Opechowski. The shifts give 2J = ?(0.55 ± 0.15) × 10?2 cm?1 for the antiferromagnetic exchange interaction among the Ni2+ ions in this crystal. The value of 2J is sub-critical in the sense that no spontaneous long-range order can be expected in this singlet-ground-state magnet.  相似文献   

6.
Pumped by the 9R(6) 12C16O2 laser lines, CH2F2 produces five SMM emissions. Levels involved in this process are identified with the help of a Fourier transform spectrum. Among these emissions we find cascade and intervibrational transitions allowed by Coriolis resonance between v9 and v3.  相似文献   

7.
New cascade laser transitions of12CH2F2 at 172.50m, 208.83m, 220.44m, 223.99m and 250.61m are reported. A waveguide FIR laser was pumped with a quasi cw12C16O2 laser operating on the 9R32 line. Together with the already known lines at 184.3m, 196.1m and 235.9m, the laser lines can be assigned to rotational transitions in the 9 vibrational band of12CH2F2 and to refill transitions of the vibrational ground state 0.  相似文献   

8.
9.
We analyze Hawking evaporation of the Callan-Giddings-Harvey-Strominger black holes from a quantum geometry perspective and show that information is not lost, primarily because the quantum space-time is sufficiently larger than the classical. Using suitable approximations to extract physics from quantum space-times we establish that (i) the future null infinity of the quantum space-time is sufficiently long for the past vacuum to evolve to a pure state in the future, (ii) this state has a finite norm in the future Fock space, and (iii) all the information comes out at future infinity; there are no remnants.  相似文献   

10.
A study is made of the effect of temperature and hydrostatic pressure on the permittivity, forbidden-band width, electrical conductivity, and photoconductivity of crystals of Sn2P2S6. The empirical results obtained indicate that a new phase-transformation line exists on the p-T diagram of Sn2P2S6 p > 0.17 GPa. This phase transformation is evidently a second-order transition, separating a paraelectric, proportionate phase from an intermediate, possibly nonproportionate phase. The results also indicate similarity of the p-T diagram of Sn2P2S6 to the x-T diagrams of ferroelectric solid solutions of Sn2P2(SexS1–x)6.Translated from Izvestiya Vysshikh Uchebnykh Zavedenii, Fizika, No. 9, pp. 57–60, September, 1985.  相似文献   

11.
A common effective action in a multidimensional R2 gravitation on a plane Rd or Rk × Td–k background is analyzed. The dependence of this effective action on the metric of the configuration space is studied.Translated from Izvestiya Vysshikh Uchebnykh Zavedenii, Fizika, No. 7, pp. 74–76, July, 1991.  相似文献   

12.
The temperature dependence of the magnetization reversal dynamics of the chiral molecular ferrimagnet [Mn{(R/S)-pn}]2[Mn{(R/S)-pn}2(H2O)][Cr(CN)6]2 has been studied at low frequencies of 1–1400 Hz, which are characteristic of the domain wall motion. It has been found from the Cole-Cole plots that domain walls undergo relaxation (at temperatures T > 10 K) and creep (at T < 10 K), and the main parameters determining these modes and the transition between them have been determined. It has been shown that the quantitative regularities of the transition between the modes of the domain wall motion correspond to the concepts of the competition between the contributions of two mechanisms to the domain wall retardation (the periodic Peierls relief and random structural defects).  相似文献   

13.
14.
15.
Muonic X-ray spectra from S and F in SF6 and H2+SF6 have been measured with a high-pressure gas target. Strong differences between the observed intensities of corresponding lines were found for the two cases. The results are compared with cascade calculations. The per-atom ratio between Coulomb capture in F and S was determined to be 0.96±0.09 and 1.04±0.24 for SF6 and H2+SF6, respectively.  相似文献   

16.
In this paper, the results of an experimental study on 6P3/2+6P3/2→7PJ+6S1/2 energy pooling collisions are presented. The experiment was carried out in cesium vapor using an external cavity diode laser for excitation under different temperatures. It is found that the response of fluorescence intensity to the frequency of excitation laser shows a two-peak phenomenon.  相似文献   

17.

Background  

We previously described planar areal differences in adult mouse visual, somatosensory, and neocortex that collectively discriminated C57BL/6J and DBA/2J inbred strain identity. Here we use a novel application of established methods of two-dimensional geometric morphometrics to examine shape differences in the cortical area maps of these inbred strains.  相似文献   

18.
The absolute integrated intensity of the 6·2μ band of NO2 at 40°C was determined from quantitative spectra at ~ 10 cm?1 resolution by the spectral band model technique. A value of 1430±300 cm?2 atm?1 was obtained. Individual line parameters, positions, intensities and ground state energies were derived, and line-by-line calculations were compared with the band model results and with the quantitative spectra obtained at ~ 0·5 cm?1 resolution.  相似文献   

19.
We consider the six dimensional hypermultiplet, vector and tensor multiplet models in (1,0) harmonic superspace and discuss the corresponding superfield actions. Manifestly a (1,0) supersymmetric procedure of computing the effective action is developed in the framework of the superfield proper-time technique. The leading low-energy contributions to the effective action is calculated.  相似文献   

20.
Ferrimagnetic resonance spectra of the [Mn{(R/S)-pn}]2[Mn{(R/S)-pn}]2(H2O)][Cr(CN)6]2 molecular magnetics were examined. It was established that, within the high-temperature (paramagnetic) region (T > 53 K), the EPR spectrum features a single Lorentz-shaped peak, while, at temperatures below 53 K, in a three-dimensional ferrimagnetic state, this peak splits into several components, some of which correspond to different states of spins in the helical chains of atoms comprising the chiral crystalline structure.  相似文献   

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