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  • 1
    Electronic Resource
    Electronic Resource
    Applications of Shape Memory Alloys in Japan (2000)
    s.l. ; Stafa-Zurich, Switzerland
    Materials science forum Vol. 327-328 (Jan. 2000), p. 17-22 
    Details
    ISSN: 1662-9752
    Source: Scientific.Net: Materials Science & Technology / Trans Tech Publications Archiv 1984-2008
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Type of Medium: Electronic Resource
    URL: http://www.tib-hannover.de/fulltexts/2011/0528/02/04/transtech_doi~10.4028%252Fwww.scientific.net%252FMSF.327-328.17.pdf
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  • 2
    Electronic Resource
    Electronic Resource
    Diagnosis and molecular analysis of an atypical case of holocarboxylase synthetase deficiency (2000)
    Springer
    European journal of pediatrics 159 (2000), S. 18-22 
    Details
    ISSN: 1432-1076
    Keywords: Key words Biotin ; Holocarboxylase synthetase ; Multiple carboxylase deficiency ; Splice error
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Holocarboxylase synthetase (HCS) deficiency is a disorder of biotin metabolism characterised by metabolic ketoacidosis and skin lesions due to reduced activities of multiple biotin-dependent carboxylases. The onset of this disease is usually between the neonatal and infantile period. Here we report the molecular analysis of an atypical case of HCS deficiency, where the patient developed his first episode of acidosis at age 8 years and had an exceptionally slow response to biotin therapy. A homozygous mutation was identified at the +5 position of the splice donor site in intron 10 of the HCS gene (IVs10 + 5(g → a)), resulting in abnormal splicing of HCS mRNA. A moderate decrease in the amount of normal HCS mRNA may account for the atypical, late-onset phenotype of this patient. Conclusion Molecular analysis is a useful tool for understanding the phenotypic variations in holocarboxylase synthetase deficiency.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050004
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  • 3
    Electronic Resource
    Electronic Resource
    Elevation of Nitric Oxide Metabolites in the Cerebrospinal Fluid of Patients with Moyamoya Disease (2000)
    Springer
    Acta neurochirurgica 142 (2000), S. 1275-1280 
    Details
    ISSN: 0942-0940
    Keywords: Keywords: Collateral circulation; intracranial-extracranial bypass surgery; moyamoya disease; nitric oxide.
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary  Background. To investigate whether nitric oxide (NO) contributes to formation of abnormal collateral circulation in patients with moyamoya disease.  Methods. Cerebrospinal fluid (CSF) samples were obtained from the subarachnoid space of the Sylvian fissure during combined bypass surgery for moyamoya disease and kept frozen until NO metabolites, nitrate and nitrite, were measured using a Griess method.  Results. Compared with control CSF obtained from 18 patients with hemifacial spasm, unruptured aneurysm, etc., concentrations of NO metabolites in 26 CSF samples of 18 patients with moyamoya disease were significantly higher (mean±SE; 17.6±1.2 vs. 10.5±1.0 μM, p〈0.01). In eight patients, the CSF samples from both the first and second operation could be obtained. NO metabolite concentrations (20.5±2.3 μM) in CSF obtained from the first surgery decreased to 15.7±1.8 μM (p〈0.01) in CSF obtained from the second, contralateral procedure. The cases of moyamoya disease with greater development of moyamoya vessels at angiographic stage 3 and 4 tended to show higher concentrations of NO metabolites than cases at earlier or later stages with a few moyamoya vessels.  Interpretation. Nitric oxide concentrations in CSF are chronically elevated in moyamoya disease, probably reflecting development of abnormal collateral circulation. i.e. moyamoya vessels. Vascular bypass surgery can reduce abnormal collateral circulation with reduced production of nitric oxide.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s007010070025
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  • 4
    Electronic Resource
    Electronic Resource
    Two-dimensional numerical equilibria of field-reversed configuration in the strong mirror field (2000)
    [S.l.] : American Institute of Physics (AIP)
    Physics of Plasmas 7 (2000), S. 4062-4069 
    Details
    ISSN: 1089-7674
    Source: AIP Digital Archive
    Topics: Physics
    Notes: Two-dimensional numerical equilibria of field-reversed configuration (FRC) plasmas in the strong mirror field applied externally are studied by means of the Grad–Shafranov equation. Appropriate choice of the pressure function is necessary to obtain a thin and elongated equilibrium, as observed in the FRC Injection Experiment (FIX) [H. Himura et al., Phys. Plasmas 2, 191 (1995)]. To solve the Grad–Shafranov equation, the finite difference method is used, applying the boundary-fitted curvilinear coordinates and the attracted grids near the separatrix. The outstanding feature of the equilibria is the presence of a narrow and sharp spike in the toroidal current profile near the separatrix. © 2000 American Institute of Physics.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1063/1.1289513
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  • 5
    Electronic Resource
    Electronic Resource
    Experimental evidence of improved confinement in a high-beta field-reversed configuration plasma by neutral beam injection (2000)
    [S.l.] : American Institute of Physics (AIP)
    Physics of Plasmas 7 (2000), S. 2294-2297 
    Details
    ISSN: 1089-7674
    Source: AIP Digital Archive
    Topics: Physics
    Notes: The first experimental result of high power (14 kV, 23 A) neutral beam (NB) injection into a high-beta field-reversed configuration (FRC) is demonstrated. The result makes it clear that the NB injection improves the plasma performance, increasing the configuration lifetime more than 200% in comparison with the ordinary FRC under similar conditions. A novel NB injection system is presented for application to FRC plasmas. A set of three concave electrodes for beam extraction is used to focus the beam enabling to pass through a narrow port. The target of beam injection is a large bore FRC plasma contained in a mirror field with a mirror ratio of 2–9. © 2000 American Institute of Physics.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1063/1.874121
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  • 6
    Electronic Resource
    Electronic Resource
    Blockade of superoxide generation prevents high-affinity immunoglobulin E receptor-mediated release of allergic mediators by rat mast cell line and human basophils (2002)
    Oxford, UK : Blackwell Science Ltd
    Clinical & experimental allergy 32 (2002), S. 0 
    Details
    ISSN: 1365-2222
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Background Previous studies have shown that rat peritoneal mast cells and mast cell model rat basophilic leukaemia (RBL-2H3) cells generate intracellular reactive oxygen species (ROS) in response to antigen challenge. However, the physiological significance of the burst of ROS is poorly understood.Objective The present study was undertaken to investigate the role of superoxide anion in mediator release in rat and human cell systems.Methods RBL-2H3 cells were directly stimulated with anti-rat FcεRI α-subunit monoclonal antibody (mAb). For the analysis of human cell system, leucocytes were isolated by dextran sedimentation from healthy volunteers or from patients, and challenged either with anti-human FcεRI mAb or with the relevant antigens. Superoxide generation was determined by chemiluminescence-based methods. The releases of histamine and leukotrienes (LT)s were determined by enzyme-linked immunosorben assay (ELISA).Results Cross-linking of FcεRI on RBL-2H3 cells or on human leucocytes from healthy donors by the anti-FcεRI mAb resulted in a rapid generation of superoxide anion, as determined by chemiluminescence using superoxide-specific probes. Similarly, leucocytes from patients generated superoxide anion in response to the challenge with the relevant allergen but not with the irrelevant allergen. Furthermore, diphenyleneiodonium (DPI), a well-known inhibitor of flavoenzymes suppressed the superoxide generation and the release of histamine and LTC4 induced by the anti-FcεRI mAb or by allergen in parallel.Conclusion These results indicate that both RBL-2H3 cells and human basophils generate superoxide anion upon FcεRI cross-linking either by antibody or by allergen challenge and that blockade of the generation prevents the release of allergic mediators. The findings strongly support the role of superoxide generation in the activation of mast cells and basophils under both physiological and pathological conditions. The findings suggest that drugs regulating the superoxide generation have potential therapeutic use for allergic disorders.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.0954-7894.2002.01263.x
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  • 7
    Electronic Resource
    Electronic Resource
    A novel homozygous mutation 371delA in TGM1 leads to a classic lamellar ichthyosis phenotype (2003)
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 148 (2003), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Malformation of the cornified cell envelope (CCE) arising from mutations of the transglutaminase (TGase) 1 gene (TGM1) is the cause of some cases of lamellar ichthyosis (LI). However, genotype/phenotype correlation in TGM1 mutations has not yet been fully clarified. We report a typical case of LI caused by a novel mutation in TGM1. The patient, a 33-year-old woman, showed thick, lamellar scales on the entire body surface. Immunofluorescence labelling with anti-TGase 1 antibodies was negative in the patient's epidermis. In situ TGase activity assay detected markedly reduced TGase activity in granular layers of the patient's epidermis. Electron microscopy revealed incomplete thickening of the CCE during keratinization in the epidermis. Sequencing of the entire exons and exon-intron borders of TGM1 revealed that the patient was a homozygote for a novel deletion mutation 371delA in exon 3. This mutation leads to a frameshift resulting in a premature termination codon 43 bp downstream from the mutation site. According to the protein modelling of TGase 1, the truncated protein from this mutated allele loses the entire catalytic core domain of TGase 1. Thus, the present homozygous mutation is expected to cause total loss of TGase 1 activity, resulting in large, dark, lamellar scales on the entire body, the classic phenotype of LI, in this patient.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2133.2003.05041.x
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  • 8
    Electronic Resource
    Electronic Resource
    Multiple actinic keratoses and squamous cell carcinomas on the sun-exposed areas of widespread vitiligo (2000)
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 142 (2000), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2133.2000.03440.x
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  • 9
    Electronic Resource
    Electronic Resource
    Comparative study of maxillary complete dentures constructed of metal base and metal structure framework (2001)
    Oxford, UK : Blackwell Science Ltd
    Journal of oral rehabilitation 28 (2001), S. 0 
    Details
    ISSN: 1365-2842
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: A removable denture designed using a three-dimensional cast metal framework (hereafter referred to as the ‘structurally designed’ denture) could extend denture longevity because it is unbreakable and easy to adjust. The aim of the present clinical study was to compare two types of maxillary removable dentures: conventional dentures and structurally designed denture. One edentulous and five partially dentate patients were fitted with two maxillary dentures made from the same impression and same occlusal relationship. About 20 days after delivery of the denture, masticatory analysis was conducted chewing phase (open, closed, and occluded); coefficients of variation and average variation were calculated. Denture vibration during tapping was then measured using an accelerometer. The patients were also interviewed about comfort, ease of chewing, speech, stability, aesthetics and preference for regular use. For both masticatory movements and denture vibration, there were no significant differences (P〉0·1) between the conventional denture and the structural design denture. In evaluating the dentures according to each criteria, the significant superiority of one denture over the other could not be determined. However, all patients subjectly preferred the structurally designed dentures for regular use. According to these findings, structurally designed dentures do not appear to have any particular physiological problems as compared with the conventional dentures.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2842.2001.00644.x
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  • 10
    Electronic Resource
    Electronic Resource
    Kerr microscopy observations of magnetization process in microfabricated ferromagnetic wires (2000)
    [S.l.] : American Institute of Physics (AIP)
    Journal of Applied Physics 87 (2000), S. 5618-5620 
    Details
    ISSN: 1089-7550
    Source: AIP Digital Archive
    Topics: Physics
    Notes: The magnetization process in microfabricated NiFe wires was observed using a Kerr microscope. Magnetic wires were made from a 20-nm-thick NiFe film by using lift-off techniques. Their width W and length L were designed as W=0.5, 1.0 and 2.0 μm and L=50 μm, respectively. One end of the wire was connected to a square shaped head with a side of 2W, which is designed to act as a domain wall source. In each wire, necks with different width of 0.2W, 0.6W, and 0.8W were introduced as artificial pinning sites of a domain wall. By using an oil-immersion lens (NA=1.3) and a Hg lamp, magnetization reversals in very narrow wires, as narrow as 0.5 μm, were clearly observed. It is confirmed that domain wall penetration, pinning, depinning, and also the direction of wall motion are controllable using square shaped head and necks with optimized width. © 2000 American Institute of Physics.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1063/1.372469
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