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81.
Hereditary argininemia manifests as neurological disturbance and mental retardation, features not observed in other amino acidemias. The cytotoxic effect of a high concentration of L-arginine (L-Arg) was investigated using NB9 human neuroblastoma cells (NB9), which express neuronal nitric oxide synthase (nNOS). When the concentration of L-Arg in the medium increased from 50 microM to 2 mM after incubation for 48 hr, the intracellular concentration of L-Arg increased from 68.0 +/- 1 pmol/10(6) cells to 1310.0 +/- 5 pmol/10(6) cells and that of L-citrulline (L-Cit) from undetectable levels to 47.1 +/- 0.2 pmol/10(6) cells (mean +/- SD of three independent analyses). This increase in intracellular L-Arg levels caused a decrease in NOS activity by approximately 71%. Flow cytometric analysis showed that reactive oxygen species (ROS) are produced in NB9 exposed to 2 mM L-Arg. The production of ROS was abolished by a NOS inhibitor, NG-nitro-L arginine-methylester. Production of ROS was also observed when NB9 were treated with L-Cit for 48 hr. To investigate the effect of L-Cit on the activity of NOS, a kinetic study on nNOS was conducted using cellular extracts from NB9. The apparent Km value of nNOS for L-Arg was 8.4 microM, with a Vmax value of 8.2 pmol/min/mg protein. L-Cit competitively inhibited NOS activity, as indicated by an apparent Ki value of 65 nM. These results suggest that L-Cit formed by nNOS in L-Arg-loaded neuronal cells inhibits NOS activity and nNOS in these L-Arg-loaded cells functions as a NADPH oxidase to produce ROS, which may cause neurotoxicity in argininemia.  相似文献   
82.
83.
Chronic granulomatous disease (CGD) of childhood is a rare inherited disease in which phagocytic cells fail to produce the normal respiratory burst in response to infectious stimuli, leaving the patient particularly susceptible to infections with bacteria and fungi that produce catalase. Between 1988 and 1993 at the NIH, 58 pulmonary cytology specimens were obtained on 24 CGD patients. The number of specimens per patient ranged from one to 13 with an average 2.4. The 58 specimens included: 33 bronchoalveolar lavages; one bronchial brushing; 20 lung or pleural mass fine-needle aspirates; three pleural fluids, and one sputum. Two lung aspirates with insufficient material, five bronchoalveolar lavages performed post-treatment to confirm clinical resolution of disease, and two bronchoalveolar lavages not submitted for culture were excluded from further analysis. Of the 49 remaining specimens obtained from patients clinically suspected of having a pulmonary infection, cytology demonstrated a pathogenic organism in nine (18%). Microbiologic cultures were positive in 19/49 (39%). Cytology identified fungus in 8/13 (62%) of documented fungal infections, including four cases where microbiology was negative. Bacterial and mixed bacterial/fungal infections were usually not detected by cytology even with appropriate strains. No organisms were identified by cytology in the four cases of nocardia or the three cases of pseudomonas infection. The combination of cytology and microbiology provided the greatest diagnostic sensitivity, yielding a diagnosis in 22/49 cases (45%). Of the 27 cases with negative cytology and microbiology, an infectious agent was identified in eight upon submission of additional material: three cytology specimens and five tissue specimens. In the remaining 19 cases, no organisms were identified, however, the patients were treated presumptively. Characteristic pathologic features of granulomatous inflammation, necrosis, and giant cells were present in fine-needle aspirates, often when on organisms could be identified, but were not seen in other types of respiratory specimens.  相似文献   
84.
The authors examine the reflectivity of the quasi-graded distributed Bragg reflector using the stopband width. The stopband width of about 1000 Å for a reflectivity of about 99% is able to be measured with an accuracy of ± 1 Å. Thus, the reflectivity is determined with high accuracy from the stopband width. It is found that the quasi-graded distributed Bragg reflector is a high-quality reflector with high reflectivity and low resistance  相似文献   
85.
InxGa1−xAs-based ohmic contacts which showed excellent contact properties for n-GaAs were demonstrated to be applicable to p-GaAs ohmic contacts. These contacts, prepared by radio-frequency sputtering, provided low contact resistance (0.2 Ω-mm), excellent thermal stability, smooth surface, and good reproducibility. The contact resistances had a weak dependence on the annealing temperatures, which was desirable in a manufacturing view point. This weak temperature dependence was explained to be due to a unique Schottky barrier height at the metal/p-InxGa1−xAs interface which does not depend on the In concentration in the InxGa1−xAs layer. The present experiment showed the possibility of simultaneous preparation of ohmic contacts for both n and p-GaAs using the same contact materials.  相似文献   
86.
87.
Switching between two cross-coupled lateral modes in gain-guided twin-stripe lasers has been experimentally observed with current injection of nominally identical pulses, which strongly indicates bistability. Near- and far-field patterns at both facets are presented, which clearly shows a skewed light field which couples from under one stripe to under the other. Cross-coupled mode operation was obtained only for relatively long cavity lasers, which is consistent with the theory for twin-stripe lasers with high interstripe gain  相似文献   
88.
Cyclic voltammograms of hexaamminecobalt complex have been measured in high pH solutions. The spectroscopic result suggested that hydroamminecobalt complex adsorbed on the electrode surface during cathodic polarization, and this species grew to form cobalt hydroxide after a long time of electrolysis. In the anodic process, the reaction orders for pH and NH3 were both |1|. Based on these results, the reaction mechanism has been discussed.The effect of light on the electrode process has been studied by irradiating with ultraviolet light of the 254 nm mercury resonance line. It has been indicated that the hydroxoammine complex is decomposed to cobalt hydroxide and free ligand under light.  相似文献   
89.
90.
The temperature dependences of some selected infrared bands of poly(vinylchloride) in the vicinity of the glass transition were examined. The intensities of 1333 and 1427 cm?1 bands changed discontinuouly at Tg; on the other hand, 2920 cm?1 did not show any significant changes at this temperature. From these results, the nature of the temperature dependence of infrared intensity of the polymer was discussed. The procedure introduced in this work offers a rapid method for the determination of the Tg as compared with the conventional infrared methods.  相似文献   
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