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131.
Huntington's disease (HD), caused by a mutation of the corresponding gene encoding the protein huntingtin (htt), is characterized by progressive deterioration of cognitive and motor functions, paralleled by extensive loss of striatal neurons. At the cellular level, pathogenesis involves an early and prolonged period of neuronal dysfunction followed by neuronal death. Understanding the molecular events driving these deleterious processes is critical to the successful development of therapies to slow down or halt the progression of the disease. Here, we examined biochemical processes in a HD ex vivo rat model, as well as in a HD model for cultured neurons using synchrotron-assisted Fourier transform infrared microspectroscopy (S-FTIRM). The model, based on lentiviral-mediated delivery of a fragment of the HD gene, expresses a mutant htt fragment in one brain hemisphere and a wild-type htt fragment in the control hemisphere. S-FTIRM allowed for high spatial resolution and distinction between spectral features occurring in gray and white matter. We measured a higher content of β-sheet protein in the striatal gray matter exposed to mutant htt as early as 4 weeks following the initiation of mutant htt exposure. In contrast, white matter tracts did not exhibit any changes in protein structure but surprisingly showed reduced content of unsaturated lipids and a significant increase in spectral features associated with phosphorylation. The former is reminiscent of changes consistent with a myelination deficiency, while the latter is characteristic of early pro-apoptotic events. These findings point to the utility of the label-free FTIRM method to follow mutant htt's β-sheet-rich transformation in striatal neurons ex vivo, provide further evidence for mutant htt amyloidogenesis in vivo, and demonstrate novel chemical features indicative of white matter changes in HD. Parallel studies in cultured neurons expressing the same htt fragments showed similar changes.  相似文献   
132.
The influence of iron species on amoxicillin (AMX) degradation, intermediate products generated and toxicity during the photo-Fenton process using a solar simulator were evaluated in this work. The AMX degradation was favored in the presence of the potassium ferrioxalate complex (FeOx) when compared to FeSO4. Total oxidation of AMX in the presence of FeOx was obtained after 5 min, while 15 min were necessary using FeSO4. The results obtained with Daphnia magna biossays showed that the toxicity decreased from 65 to 5% after 90 min of irradiation in the presence of FeSO4. However, it increased again to a maximum of 100% after 150 min, what indicates the generation of more toxic intermediates than AMX, reaching 45% after 240 min. However, using FeOx, the inhibition of mobility varied between 100 and 70% during treatment, probably due to the presence of oxalate, which is toxic to the neonates. After 240 min, between 73 and 81% TOC removal was observed. Different pathways of AMX degradation were suggested including the opening of the four-membered β-lactamic ring and further oxidations of the methyl group to aldehyde and/or hydroxylation of the benzoic ring, generating other intermediates after bound cleavage between different atoms and further oxidation to carboxylates such acetate, oxalate and propionate, besides the generation of nitrate and ammonium.  相似文献   
133.
Degradation of the sulfonamide sulfamethazine (SMZ) by the white-rot fungus Trametes versicolor was assessed. Elimination was achieved to nearly undetectable levels after 20 h in liquid medium when SMZ was added at 9 mg L− 1. Experiments with purified laccase and laccase-mediators resulted in almost complete removal. On the other hand, inhibition of SMZ degradation was observed when piperonilbutoxide, a cytochrome P450-inhibitor, was added to the fungal cultures. UPLC-QqTOF-MS analysis allowed the identification and confirmation of 4 different SMZ degradation intermediates produced by fungal cultures or purified laccase: desulfo-SMZ, N4-formyl-SMZ, N4-hydroxy-SMZ and desamino-SMZ; nonetheless SMZ mineralization was not demonstrated with the isotopically labeled sulfamethazine-phenyl-13C6 after 7 days. Inoculation of T. versicolor to sterilized sewage sludge in solid-phase systems showed complete elimination of SMZ and also of other sulfonamides (sulfapyridine, sulfathiazole) at real environmental concentrations, making this fungus an interesting candidate for further remediation research.  相似文献   
134.
The CALIOPE project, funded by the Spanish Ministry of the Environment, aims at establishing an air quality forecasting system for Spain. With this goal, CALIOPE modeling system was developed and applied with high resolution (4 km × 4 km, 1 h) using the HERMES emission model (including emissions of resuspended particles from paved roads) specifically built up for Spain. The present study provides an evaluation and the assessment of the modeling system, coupling WRF-ARW/HERMES/CMAQ/BSC-DREAM8b for a full-year simulation in 2004 over Spain. The evaluation focuses on the capability of the model to reproduce the temporal and spatial distribution of gas phase species (NO2, O3, and SO2) and particulate matter (PM10) against ground-based measurements from the Spanish air quality monitoring network. The evaluation of the modeling results on an hourly basis shows a strong dependency of the performance of the model on the type of environment (urban, suburban and rural) and the dominant emission sources (traffic, industrial, and background). The O3 chemistry is best represented in summer, when mean hourly variability and high peaks are generally well reproduced. The mean normalized error and bias meet the recommendations proposed by the United States Environmental Protection Agency (US-EPA) and the European regulations. Modeled O3 shows higher performance for urban than for rural stations, especially at traffic stations in large cities, since stations influenced by traffic emissions (i.e., high-NOx environments) are better characterized with a more pronounced daily variability. NOx/O3 chemistry is better represented under non-limited-NO2 regimes. SO2 is mainly produced from isolated point sources (power generation and transformation industries) which generate large plumes of high SO2 concentration affecting the air quality on a local to national scale where the meteorological pattern is crucial. The contribution of mineral dust from the Sahara desert through the BSC-DREAM8b model helps to satisfactorily reproduce episodic high PM10 concentration peaks at background stations. The model assessment indicates that one of the main air quality-related problems in Spain is the high level of O3. A quarter of the Iberian Peninsula shows more than 30 days exceeding the value 120 μg m−3 for the maximum 8-h O3 concentration as a consequence of the transport of O3 precursors downwind to/from the Madrid and Barcelona metropolitan areas, and industrial areas and cities in the Mediterranean coast.  相似文献   
135.
A method of forming cubic phase (zinc blende) GaN (referred as c‐GaN) on a CMOS‐compatible on‐axis Si (100) substrate is reported. Conventional GaN materials are hexagonal phase (wurtzite) (referred as h‐GaN) and possess very high polarization fields (~MV/cm) along the common growth direction of <0001>. Such large polarization fields lead to undesired shifts (e.g., wavelength and current) in the performance of photonic and vertical transport electronic devices. The cubic phase of GaN materials is polarization‐free along the common growth direction of <001>, however, this phase is thermodynamically unstable, requiring low‐temperature deposition conditions and unconventional substrates (e.g., GaAs). Here, novel nano‐groove patterning and maskless selective area epitaxy processes are employed to integrate thermodynamically stable, stress‐free, and low‐defectivity c‐GaN on CMOS‐compatible on‐axis Si. These results suggest that epitaxial growth conditions and nano‐groove pattern parameters are critical to obtain such high quality c‐GaN. InGaN/GaN multi‐quantum‐well structures grown on c‐GaN/Si (100) show strong room temperature luminescence in the visible spectrum, promising visible emitter applications for this technology.  相似文献   
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This report summarizes the findings and recommendations of the second Committee of Visitors (COV) whose charge was to review the manner in which the U. S. Department of Energy’s Office of Fusion Energy Science (OFES) manages certain programs under its charter. The specific programs reviewed by this COV involve confinement innovation and basic plasma sciences. The charge letter from the Department of Energy is included as Appendix A.  相似文献   
139.
We present 11 lymphoceles in 180 consecutive kidney transplants (6%). We study the potential etiologic factors, symptoms, diagnostic evaluation and treatment, stressing the role of laparoscopy in management of bulky and loculated symptomatic lymphoceles. This technique allows to reduce morbidity, increasing efficacy and shortening hospitalization.  相似文献   
140.
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