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51.
In 2010, a new recessive cohesinopathy disorder, designated Warsaw breakage syndrome (WABS), was described. The individual with WABS displayed microcephaly, pre- and postnatal growth retardation, and abnormal skin pigmentation. Cytogenetic analysis revealed mitomycin C (MMC)-induced chromosomal breakage; however, an additional sister chromatid cohesion defect was also observed. WABS is genetically linked to bi-allelic mutations in the ChlR1/DDX11 gene which encodes a protein of the conserved family of Iron–Sulfur (Fe–S) cluster DNA helicases. Mutations in the budding yeast ortholog of ChlR1, known as Chl1, were known to cause sister chromatid cohesion defects, indicating a conserved function of the gene. In 2012, three affected siblings were identified with similar symptoms to the original WABS case, and found to have a homozygous mutation in the conserved Fe–S domain of ChlR1, confirming the genetic linkage. Significantly, the clinically relevant mutations perturbed ChlR1 DNA unwinding activity. In addition to its genetic importance in human disease, ChlR1 is implicated in papillomavirus genome maintenance and cancer. Although its precise functions in genome homeostasis are still not well understood, ongoing molecular studies of ChlR1 suggest the helicase plays a critically important role in cellular replication and/or DNA repair.  相似文献   
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The sol–gel auto-combustion method was adopted to synthesize nanomaterials of single-phase X-type hexagonal ferrites with the composition of Sr2?xGdxNi2Fe28?yCdyO46 (x=0.00, 0.02, 0.04, 0.06, 0.08, 0.10 and y=0, 0.1, 0.2, 0.3, 0.4, 0.5). The structural properties were carried out by XRD analysis and the lattice parameters show variation with the doping of Gd–Cd. The average particle size measured by TEM was in the range of 8–10 nm which is beneficial in obtaining suitable signal-to-noise ratio in recording media and biomedical applications. The room temperature resistivity enhanced with the increase of the dopant concentration. The increase in resistivity indicates that the synthesized materials can be considered good for the formation of the multilayer chip inductors (MLCIs) as well as for the reduction of eddy current losses. The dielectric constant decreased with the increase in the frequency which is the general reported trend of the hexagonal ferrites and can be explained on the basis of Koop?s theory and Maxwell–Wagner polarization-model. The abnormal dielectric behavior indicates the formation of small polarons in the material. The maximum value of tangent loss at low frequencies reflects the application of these materials in medium frequency devices (MF).  相似文献   
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Mechanism of the Pasteur effect   总被引:6,自引:0,他引:6  
J Barker  M A Khan  T Solomos 《Nature》1966,211(5048):547-548
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The effects of composition and thermal annealing in between glass transition and crystallization temperature on the optical and structural properties of Ga25Se75-xTex were investigated. The glass transition and crystallization temperature of the synthesized samples was measured by non-isothermal DSC measurements. Amorphous thin films of Ga25Se75-xTex glasses were grown onto ultra clean glass/Si wafer (100) substrates using the vacuum evaporation technique. The effect of thermal annealing on the optical gap (Eg) for Ga25Se75-xTex thin films in the temperature range 358-388K is studied. As-prepared and annealed thin films were characterized by X-ray diffraction, field emission scanning electron microscopy, energy dispersive X-ray spectroscopy and optical absorption. Thermal annealing was found to be accompanied by structural effects, which in turn, lead to changes in the optical constants. The optical absorption coefficient (a) for as-deposited and thermally annealed films was calculated from the absorbance data. From the knowledge of absorption coefficient at different wavelengths, the optical band gap (Eg) was calculated for all compositions of Ga25Se75-xTex thin films before and after thermal annealing. Results indicate that allowed indirect optical transition is predominated in as-deposited and thermally annealed thin films. The influence of Te incorporation and thermal annealing in Ga25Se75-xTex thin films results in a gradual decrease in the indirect optical gap, this behaviorcan be explained as increased tailing. The decrease in optical band gap and an increase in absorption coefficient and extinction coefficient with thermal annealing can be attributed to transformation from amorphous to crystalline phase.  相似文献   
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The structural and magnetic properties of essentially phase pure AlFe_2B_2 prepared by arc melting were compared with a sample containing impurities. Analysis was carried out by means of synchrotron X-ray diffraction, magnetic measurements and M?ssbauer spectroscopy. The analysis of synchrotron X-ray diffraction data confirmed the orthorhombic structure of space group Cmmm for AlFe_2B_2 with Al_(13)Fe_4 as main impurity phase. The magnetic measurements revealed an unsaturated ferromagnetic state in AlFe_2B_2 with a transition temperature(T_c) of 286 K. Isothermal magnetization measurment at 5 K gave a saturation magnetization of 0.7 μB per Fe atom while Arrott plots establish the second order nature of ferromagnetic transition. The thermal evolution of M?ssbauer spectra confirmed the ferromagnetic nature of this material revealing an hyperfine field of 73 kOe and an isomer shift of 0.46 mm/s at 100 K. The M?ssbauer spectra of a phase pure sample was compared with the one containing impurity phases. It is suggested that the low temperature paramagnetic contribution in M?ssbauer spectra of phase pure material may have its origin in some intrinsic phenomena arising from defects or inhomogeneities in crystal structure.  相似文献   
59.
A thermally grown oxide layer formed by hot corrosion was investigated as an interface between plasma-sprayed coatings and a nickel-based superalloy substrate. The hot corrosion mechanism of NiCr-Cr2O3 and Al2O3-40wt% TiO2 (A40T) plasma coated Inconel 617 was evaluated. The experiments were carried out at 1000℃ using a combination of Na2SO4, NaCl, and 2O5 salts to simulate the conditions of a gas turbine in a marine environment. The hot corrosion results revealed the spallation and dissolution of oxides upon prolonged exposure. Optical images and scanning electron micrographs of the exposed samples revealed the formation of oxide scale and provided details of its morphology in NiCr-Cr2O3 coated samples. Microstructure characterization of A40T coatings demonstrated a thermally grown oxide (TGO) layer at 1000℃. Increasing the thickness of the TGO layer decreased the corrosion resistance. The elemental analysis and image mapping revealed the migration of active elements from the substrate and coatings toward the corrosive environment.  相似文献   
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In this study,spatial analysis techniques were used to generate climatic zoning maps of the East Murrumbidgee Irrigation Area (MIA),Australia.Long term climate data were used to analyze and assess regional climatic variation by cluster analysis in a GIS environment.Based on thermal and moisture factors,four variables were chosen to develop climate zones.These variables include annual average rainfalls,annual average ETc,annual average Growing Day Degree (GDD) and daily average temperature generated from daily climate data of 36 years (1971-2006) on 12 local weather stations as the fundamental elements of regional climate characteristics.GIS-based spatial analysis models are used to map climate sub-regions from sets of climatic parameters derived from regional climate data sources.Two climate zoning schemes are presented by integration of spatial interpolation,spatial cluster analysis and climate indices methods.One scheme comes with the two climate zones of the West and the East based on spatial cluster analysis according to selected climatic variables,and the other comes with the three comprehensive hydro-thermal zones and the six hydro-thermal balance zones.  相似文献   
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