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  • 1
    ISSN: 1365-2230
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary Dystrophic epidermolysis bullosa (DEB) is caused by mutations in the COL7A1 gene encoding type VII collagen, the major component of anchoring fibrils. The characteristic genetic lesion in dominant DEB (DDEB) is a glycine substitution in the collagenous domain of the protein. In this study, we identified a Chinese family with a four-generation pedigree of DDEB, in whom a novel glycine substitution mutation in COL7A1 was demonstrated. A heterozygous nucleotide G→A transition at position 6208 in exon 74 of COL7A1 was detected, which resulted in a glycine to arginine substitution (G2070R) in the triple-helical domain of type VII collagen. This substitution was not found in 110 unrelated normal alleles. This report emphasizes the predominance of glycine substitution mutations in DDEB and contributes to the expanding database on COL7A1 mutations.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    [S.l.] : American Institute of Physics (AIP)
    Journal of Applied Physics 76 (1994), S. 3313-3320 
    ISSN: 1089-7550
    Source: AIP Digital Archive
    Topics: Physics
    Notes: Surface preflashover phenomena across alumina ceramics in vacuum are presented. The localized plasmas are identified by the spatially resolved images that appear in a pulse surface preflashover phase using an intensified charge-coupled-device camera, along with coordinated, time-resolved preflashover current and luminosity. It was observed that significant preflashover activity associated with the localized plasmas on the surface appears after a flashover event and disappears after a few pulse voltage applications. Large bursts of current pulses (several hundred milliamperes) with no counterpart in the luminosity signal were also observed during preflashover. These phenomena cannot be explained satisfactorily by electron stimulated gas desorption. The effect of adsorbed gases and surface polishing on preflashover are presented and discussed. The experimental results indicate that preflashover essentially depends on the surface state (physical and chemical) of the solid insulator. The preflashover conduction associated with the localized plasmas is attributed to the detrapping and impact-ionization processes associated with defect centers. The results reported support the surface flashover model based on trapping/detrapping and impact ionization processes.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1089-7550
    Source: AIP Digital Archive
    Topics: Physics
    Notes: The surface roughness of polished InP (001) wafers were examined by x-ray reflectivity and crystal truncation rod (CTR) measurements. The root-mean-square roughness and the lateral correlation scale were obtained by both methods. The scattering intensities in the scans transverse to the specular reflection rod were found to contain two components. A simple surface model of surface faceting is proposed to explain the experimental data. The sensitivities of the two methods to the surface structure and the role of the resolution functions in the CTR measurements are discussed.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1365-3040
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Biology
    Notes: After exposure to a doubled CO2 concentration of 750 µL L−1 for 2 months, average relative growth rate (RGR) of Mokara Yellow increased 25%. The two carboxylating enzymes, ribulose-1,5-bisphosphate carboxylase/oxygenase (Rubisco) and phosphoenolpyruvate carboxylase (PEPCase), responded differently to CO2 enrichment. There was a significant daytime down-regulation in Rubisco activity in the leaves of CO2-enriched plants. However, PEPCase activity in CO2-enriched plants was much higher in the dark period, although it was slightly lower during the daytime than that at ambient CO2. Leaf sucrose–phosphate synthase (SPS) and sucrose synthase (SS) activities in CO2-enriched plants increased markedly, along with a night-time increase in total titratable acidity and malate accumulation. There was a remarkable increase in the levels of indole-3-acetic acid (IAA), gibberellins A1 and A3 (GA1+3), isopentenyladenosine (iPA) and zeatin riboside (ZR) in the expanding leaves of plants grown at elevated CO2. It is suggested that (1) the down-regulation of Rubisco and up-regulation of SPS and SS are two important acclimation processes that are beneficial because it enhanced both photosynthetic capacity at high CO2 and reduced resource investment in excessive Rubisco capacity; (2) the increased levels of plant hormones in CO2-enriched M. Yellow might play an important role in controlling its growth and development.
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  • 5
    Electronic Resource
    Electronic Resource
    Woodbury, NY : American Institute of Physics (AIP)
    Applied Physics Letters 65 (1994), S. 3317-3319 
    ISSN: 1077-3118
    Source: AIP Digital Archive
    Topics: Physics
    Notes: We have studied the surface scattering of x rays from mechanical-chemical polished InP (001) wafers with sulfur and/or iron doping. The scattering intensities in the scans transverse to the specular reflection rod were found to contain two components. A simple surface model was proposed to explain the experimental data. The results were also compared with those obtained from crystal truncation rod measurements. © 1994 American Institute of Physics.
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  • 6
    Electronic Resource
    Electronic Resource
    [S.l.] : American Institute of Physics (AIP)
    Journal of Applied Physics 72 (1992), S. 3474-3479 
    ISSN: 1089-7550
    Source: AIP Digital Archive
    Topics: Physics
    Notes: Si1−xGex/Si strained-layer superlattices grown by molecular-beam epitaxy on Si substrates were investigated by x-ray double-crystal diffraction and x-ray grazing incidence diffraction. Both coherent and incoherent interfaces between the two components of the superlattices were observed. By fitting computer-simulated double-crystal x-ray-diffraction rocking curves to the experimental data, it is determined that there exist graded variations in both the component thickness ratio t1/t2 (t1 and t2 are the thickness of the Si1−xGex and the Si layers, respectively) and the fraction x in one sample. The x-ray grazing incidence diffraction experiments reveal a lattice strain relaxation of about 27% in another sample. The lattice relaxation and the influence of variations of x and t1/t2 on the rocking curves are discussed.
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  • 7
    Electronic Resource
    Electronic Resource
    [S.l.] : American Institute of Physics (AIP)
    Journal of Applied Physics 82 (1997), S. 2281-2287 
    ISSN: 1089-7550
    Source: AIP Digital Archive
    Topics: Physics
    Notes: The surface and interface structures of ZnTe epilayers grown by molecular beam epitaxy on GaSb (001) substrates under different conditions have been investigated by high resolution x-ray diffraction and grazing incidence scattering. Reciprocal space mapping around the symmetrical diffraction reciprocal point 004 and asymmetrical diffraction point 1¯1¯5 showed that the ZnTe epilayers, in the samples investigated, were fully strained to the substrate. The crystalline quality of the ZnTe epilayer grown on a substrate annealed in a Zn flux was very good, while evidence for an interfacial layer, of thickness varying from 2–20 nm, was found when the substrate was annealed in a Te flux prior to growth. This is attributed to Ga2Te3 formation at the interface. The interfacial layer roughens the interface and surface, and both crystal truncation rod measurements and grazing incidence x-ray reflectivity show the surface roughness to be about 4 nm. Such a rough surface and interface is also inferred from the broader distribution along the transverse direction in reciprocal space maps. A shorter lateral correlation length is found for the roughness of the sample containing the interfacial layer. The disappearance of interference fringes is attributed to nonuniformity of the interfacial layer. © 1997 American Institute of Physics.
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