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1.
Ambiguous abdominal situs, asplenia/polysplenia and severe cardiac malformations characterize heterotaxy in humans. These anomalies result from the inability of the developing embryo to establish normal left-right asymmetry. We have studied an interesting family in which the heterotaxy phenotype segregates as an X-linked recessive trait. In order to map the heterotaxy locus (HTX), we have analysed 39 family members using highly-polymorphic microsatellite markers from the X chromosome. One of these markers, DXS994, shows no recombination with the disease locus in 20 informative meioses. Linkage analysis results in a maximum lod score of 6.37. Current genetic and physical mapping data assign the order of loci in Xq24-q27.1 as cen-DXS1001-(DXS994, HTX)-DXS984-tel. These results establish the first mapping assignment of situs abnormalities in humans.  相似文献   
2.
Kenta Suzuki  Masatoshi Tokita 《Polymer》2005,46(19):8313-8320
We investigated liquid crystallization of liquid crystalline polyester BB-5 during isothermal annealing by digital high-fidelity microscope and light scattering. A liquid crystalline spherical domain having a radius of micrometers appeared by annealing at around 180 °C. The domain grew dendritically in all directions. Neighboring liquid crystalline regions coalesced and then interconnected. The interconnected structure changed to a co-continuous two-phase structure with increasing ordering of the liquid crystalline phase, and the interface between the liquid crystalline phase and the isotropic phase became smoother over time. Liquid crystallization stopped before volume filling the whole space, and the liquid crystalline phase and isotropic phase coexisted. The liquid crystalline region became narrower with an increase in the temperature of the liquid crystallization. Such structural development is different from the liquid-liquid phase separation via spinodal decomposition, and it may be attributed to the segregation of non-liquid crystallizable low molecular weight molecules from the growth front by fractionation of the molecular weight distribution during the liquid crystallization in terms of the instability of the diffusion-controlled interface.  相似文献   
3.
A 40-year-old man with epidermodysplasia verruciformis showed a decrease in peripheral blood T cells and abnormal expansion of large granular lymphocytes, accompanied by increased natural killer cell activity. Surface marker analysis of his large granular lymphocytes demonstrated that the subset, CD 57+ and CD 16+, had increased. His father, who had no skin lesions of epidermodysplasia verruciformis, displayed similar blood changes and his brother showed a decrease in T cells and a slight increase in CD 16+ natural killer cells, whereas his mother revealed only a slight decrease in T cells. Our present study indicates that epidermodysplasia verruciformis might be associated with hereditary abnormal expansion of large granular lymphocytes and a decrease in T cells.  相似文献   
4.
A bucket-type high-density (0.25-1.2-mA/cm2) low-energy (500-2000 V) ion source was utilized for high-speed phosphorus doping directly into a thin polysilicon layer without cap SiO2. Doping gas with He dilution was selected to reduce etching of polysilicon film. Excimer laser (XeCl, 8 mm×8 mm) pulse annealing was introduced to activate effectively the doped impurity. The combination of these techniques provided a practically low sheet resistance for the TFT source, drain, and gate with a short time doping. The low-temperature polysilicon TFT fabricated with a doping time of 10 s had characteristics comparable to those of that fabricated by a longer time doping or conventional ion implantation, showing the practicality of this technology and its promise for giant microelectronics  相似文献   
5.
Superconductivity in a tungsten-containing carbon-oxide film was reported. The film with 500 nm thickness was deposited onto polycrystalline silicon oxides using chemical vapor deposition and the co-sputtering of a tungsten metal target. The bonding state of the carbon atoms and the macroscopic and microscopic crystal structure of the film were investigated by Raman spectroscopy, X-ray diffraction and transmission electron microscopy measurements. From the experimental results, we determined that this film essentially had an amorphous structure. The temperature dependence on resistivity was measured in the temperature range of 2–300 K. Resistive superconducting transition was observed at 3.8 K. The dc magnetizations were measured in the temperature range of 1.8–6.5 K. The diamagnetism resulting from a superconductive state was observed below 3.75 K, which is consistent with a resistive superconducting transition. It is thought that the finite sized clusters of the different superconductive transition temperatures cooperatively produce a macroscopic superconducting phenomenon.  相似文献   
6.
To investigate the mechanism of high elongation of natural rubber attained by the ‘racking method’, a strip of smoked-sheet was elongated up to 150 times in length, by stretching rapidly at room temperature and forcing to shrink at 75-80 °C repeatedly. On X-ray diffraction, a typical fibre pattern with an amorphous ring appeared already at ×10 (stretch ratio=10). The degree of crystallinity increased to the level of 17-18% at ×20 and turned to decrease after ×60, but the degree of crystallite orientation reached at a high level already at ×10 and did not change significantly and the half-height width of reflection profiles stayed almost at a constant level, while the Young's modulus increased up to ca. 300 MPa at ×100 and then fell rather discontinuously. It was assumed that some fractions of chain segments between entanglements were gradually broken in each step of stretching operation, rather than disentangled and slipped.  相似文献   
7.
Radiation damages due to 8 MeV electron irradiation in electrical properties of CuInSe2 thin films have been investigated. The n-type CuInSe2 films in which the carrier concentration was about 3×1016 cm−3, were epitaxially grown on a GaAs(0 0 1) substrate by RF diode sputtering. No significant change in the electrical properties was observed under the electron fluence <3×1016 e cm−2. As the electron fluence exceeded 1017 e cm−2, both the carrier concentration and Hall mobility slightly decreased. The carrier removal rate was estimated to be about 0.8 cm−1, which is slightly lower than that of III–V compound materials.  相似文献   
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Cognitive model of team cooperation in en-route air traffic control   总被引:1,自引:1,他引:0  
Since controller teams are in charge of en-route air traffic control, team cooperation is a key issue for good control performance. We conducted ethnographic field observation at the Tokyo Area Control Center and then analyzed the obtained data to develop a cognitive model of team cooperation in en-route air traffic control. We segmented conversational records, behavioral records, and so on by control unit, and then clarified relations between the segments and identified expert knowledge and judgment behind them. Cognitive processes of controller teams were reconstructed based on a concept of distributed cognition. The analysis revealed that the mutual belief model is applicable to team cooperation processes, the role assignment of tasks within a controller team is implicit and that control plans are implemented smoothly once team situation awareness has been established. A cognitive model of controllers?? team cooperation has been constructed based on these findings.  相似文献   
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