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The molecular basis for a hereditary type I protein S (PS) deficiency was investigated. DNA sequence analysis in the proband showed a novel missense mutation substituting Cys (TGT) for Arg474 (CGT) that is a highly conserved amino acid residue among the related proteins. This missense mutation cosegregated with the type I PS deficiency in this family. Transient expression studies showed that the secretion of the recombinant Cys-mutant PS was markedly decreased compared with that of the recombinant wild-type PS, reproducing the observed phenotype of type I deficiency. Stable expression and pulse-chase experiments demonstrated an intracellular degradation and an impaired secretion of the recombinant Cys-mutant PS. Furthermore, the substitution of Arg474 by Ala or Glu, but not by Lys, markedly reduced the secretion of the recombinant PS mutants in transient expression studies, suggesting that a positively charged basic amino acid might be needed at residue 474 and might play a key role in the protein structure and conformation of the sex hormone binding globulin-homology domain of the PS molecule. We postulate that the loss of the highly conserved Arg474 might be responsible for the type I PS deficiency inherited in this family.  相似文献   
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Two kinds of TV cameras have been developed that are capable of showing the wavelengths of ultraviolet, infrared, and visible light by means of a false color method. One is sensitive to wavelengths from 250 to 650 nm (ultraviolet to yellow). It can simulate the color vision of the honey bee and other insects, reproducing the nectar guide of a flower or displaying the sex of a cabbage white moth, which is indistinguishable to the naked eye. The other is sensitive to a wider wavelength of 250 to 1,050 nm (ultraviolet to near infrared). According to the filter selected, it obtains not only visual images of invisible light information but also pictures same as those from an ordinary color camera. These TV cameras can be applied in many fields, including science, art, medicine, and industry  相似文献   
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A potential barrier model along the grain boundaries in ultrathin tin dioxide (SnO2) gas sensors is presented. It is assumed that the negatively charged oxygens are adsorbed only on the grain boundaries. The potential barrier depth is expressed as functions of grain size, donor concentration and surface coverage of adsorbed oxygen ions at the boundaries. A direct consequence is that the conduction electrons are effectively confined in a grain when the film thickness becomes smaller than a critical value. This indicates a drastic increase in resistivity with decreasing film thickness in air, and thus an occurrence of an extremely high gas sensitivity.  相似文献   
15.
Takuda  H.  Yamazaki  N.  Hatta  N.  Kikuchi  S. 《Journal of Materials Science》1995,30(4):957-963
Using an Al-Mg-Cu alloy developed for auto body panels, strip sheets are experimentally produced by various cold-rolling and annealing procedures. Tensile and metallographic properties of the sheets and their relations are examined to attain high formability. The elongation is closely related to the grain size, and increases with the final annealing temperature. The rolling texture influences the plastic anisotropy, the Lankford value of the sheets. The comparatively high Lankford values are obtained under the condition that both the intermediate and the final annealing temperatures are higher and the reduction ratio after the intermediate annealing is smaller.  相似文献   
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This 512 Kw×8 b×3 way synchronous BiCMOS SRAM uses a 2-stage wave-pipeline scheme, a PLL self-timing generator and a 0.4-μm BiCMOS process to achieve 220 MHz fully-random read/write operations with a GTL I/O interface. Newly developed circuit technologies include: 1) a zig-zag double word-line scheme, 2) a centered bit-line load layout scheme, and 3) a phase-locked-loop (PLL) with a multistage-tapped ring oscillator which generates a clock cycle proportional pulse (CCPP) and a clock edge lookahead pulse (CELP)  相似文献   
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A series of mixtures of Japanese subbituminous Taiheiyo coal and Athabasca oil sand bitumen (AOB) with various coal concentrations (0–100 wt%) was coprocessed in a 70 ml autoclave at 420°C for 1 h in the presence of H2 (50 kg/cm2 at room temperature) and sulfided Ni---Mo/Al2O2 catalyst. The mixture containing 2 wt% coal produced the largest amount of hexane soluble fraction (HS) and the smallest amount of benzene insoluble fraction (BI). Thus, a synergistic liquid production occurred for this mixture with 2 wt% coal by suppressing the retrogressive reactions which proceeded for pure AOB. The HS obtained from mixtures with 2–30 wt% showed higher H/C ratios and lower heteroatom contents than those obtained from pure AOB and the mixtures with more than 30 wt% coal. The amounts of transferable hydrogen contained in the mixtures were estimated using anthracene as a hydrogen acceptor. The mixtures with 2–10 wt% coal contained higher amounts of donor hydrogen than pure AOB. The HS yield from the various mixtures was correlated with the amount of donor hydrogens contained in the mixtures, except for the mixture with 10 wt% coal. Thus, the important factor which results in synergism is suggested to be the amount of donor hydrogens contained in the feed mixtures.  相似文献   
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The structural change of diamond induced by hydrogen plasma exposure at room temperature, and its thermal stability, were investigated using electron spin resonance (ESR), X-ray photoelectron spectroscopy (XPS) and Fourier transform infrared spectroscopy (FTIR) techniques. ESR observation revealed that it gave rise to a highly defective structure (spin concentration of 1020 cm− 3), which is very similar to the structure of hydrogenated amorphous carbon (a-C : H) confirmed by XPS and FTIR. Post-annealing was also carried out to clarify the thermal stability of the defects. The number of spin centers decreased with increasing annealing temperature, and eventually, the defective structure changed to a graphitic one by annealing at 800 °C.  相似文献   
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