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  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 148 (2003), S. 0 
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary Background White sponge naevus (WSN) is a rare, autosomal dominant disorder that predominantly affects noncornified stratified squamous epithelia, most commonly the buccal mucosa. Clinically, WSN manifests as thickened spongy mucosa with a white opalescent tint in the mouth and may be confused with other disorders that cause white lesions on oral mucosa. Recent studies have identified pathogenic mutations in KRT4 and KRT13, the genes encoding mucosa-specific keratins, in WSN. Objectives To search for possible mutations in KRT4 and KRT13. Methods We report a case of WSN in a young man who presented with diffuse irregular whitish plaques involving the buccal and gingival mucosae and the tongue. Results Pathologically, the affected mucosa showed epithelial thickening, parakeratosis and extensive vacuolization of the suprabasal keratinocytes. Mutation analysis revealed a heterozygous missense mutation 1345G→A in KRT4, predicting an amino acid change, E449K, in the 2B domain of the K4 polypeptide. Conclusions We report the first mutation analysis of a Taiwanese patient with WSN. Potentially this novel mutation could disrupt the stability of keratin filaments and result in WSN.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 146 (2002), S. 0 
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary Background Hailey–Hailey disease (HHD) is an autosomal dominant disorder with recurrent eruption of vesicles and bullae involving predominantly the neck, groin and axillary regions. Histopathology shows suprabasal cleavage in epidermal cells. Recent studies have revealed that HHD is caused by mutations in the ATP2C1 gene encoding a novel Ca2+ pump. Objectives To analyse the mutations of the ATP2C1 gene in Taiwanese patients with HHD. Methods In total, five familial and two sporadic cases of HHD were retrieved from the medical records. The diagnosis of HHD was made based on the characteristic clinical features and histopathological evidence. All 27 exons and flanking intron boundaries were amplified by polymerase chain reaction and products analysed by direct sequencing. Results We identified six novel mutations and one reported mutation: three deletion mutations (nt884–904del, 1459delCTCA, 1975delA), two non-sense mutations (R39X, R783X), one mis-sense mutation (A730T) and one splicing mutation (483 + 2T→A). The non-sense mutation R39X had been reported previously; the other six mutations are novel mutations. Conclusions These results demonstrate that a spectrum of ATP2C1 gene mutations is present in Taiwanese HHD patients.
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  • 3
    ISSN: 1365-2230
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Infantile systemic hyalinosis (ISH) is a very rare infantile stiff-skin syndrome characterized by extensive deposits of hyaline material in various organs, especially the skin and gingiva. Recent studies identified pathogenic mutations in the capillary morphogenesis gene 2 (CMG2) in both ISH and juvenile hyaline fibromatosis (JHF). Capillary morphogenesis protein-2 is an integrin-like cell surface receptor for laminins and type IV collagen, and may play a key role in cell–matrix or cell–cell interactions. We report a case of ISH in a 13-month-old Taiwanese girl who manifested progressive joint contractures, recurrent chest infections, chronic diarrhoea with severe hypoalbuminemia and ascites, gum hypertrophy, and violaceous papules and nodules over the occipital area, neck, lumbosacral and anogenital areas since birth. Skin biopsy revealed a thickened and hyalinized papillary dermis. Electron microscopy showed abundant extracellular fibrillogranular material and active fibroblasts with conspicuous Golgi complex filled with fibrillar material. Mutation analysis identified a homozygous 1073–1074insC mutation of CMG2 which had been reported in four other families and may represent a mutation hot spot.
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  • 4
    ISSN: 1420-9071
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary The structure 3-butyl-5-propylindolizidine (2), tentatively assigned to a minor alkaloid in skin extracts from a number of poison frogs of the Neotropical genusDendrobates, has been confirmed and its stereochemistry determined as 5E, 9E (2d) by comparison on GC and GC-MS with the four synthetic diastereomers2a-2d.
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  • 5
    ISSN: 1420-9039
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mathematics , Physics
    Description / Table of Contents: Zusammenfassung Das elastodynamische Spannungsgebiet um einen pfennigförmigen Bruchriss, der mit konstanter Geschwindigkeit in einem unendlichen Körper läuft, wird als Summe der verbundenen statischen Lösung und der Wellenwirkungsterme aufgefasst. Die Ergebnisse schliessen das Spannungsgebiet im Bereich des laufenden Bruchrisses und die zugehörigen statischen Lösungen als Sonderlösungen ein. Die Ringspannung der Risspitze, πθθ wird als Funktion der RissgeschwindigkietV, der Geschwindigkeit der Scherungswellec 2, der Poissonzahl ν und des Winkels θ zwischen der Bruchebene und einer Radiallinie von der Risspitze aus ausführlich studiert. Die Theorie sagt aus, dass der laufende Riss bei der kritischen Geschwindigkeit von 0.665c 2 in der Richtung θ=41° für ν=0.25 zu gabeln anfangen kann. Der vorausgesagte Gabelungswinkel wird mit Versuchserfahrungen verglichen.
    Notes: Abstract The elastodynamic stress field around a penny-shaped crack running at a constant speed in an infinite solid is obtained as the sum of the associated static solutions and the wave-effect terms. The results include the stress field in the plane of the running crack and the associated static solutions as special solutions. The crack-tip circumferential stress, πθθ, is studied in detail as a function of the crack speed,V, the shear wave speed,c 2, Poisson's ratio, ν, and the angle, θ, between the crack plane and a crack-tip radial line. The theory predicts that the running crack may start to bifurcate at the critical speed of 0.665c 2 in the direction θ=41° for ν=0.25. The bifurcation angle predicted is compared to an experimental observation.
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  • 6
    ISSN: 1619-6937
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics , Physics
    Notes: Summary Fracture of a hybrid laminate containing a pair of collinear cracks in the central layer is studied. The surfaces of the cracks are opened by an arbitrary opening pressure. The problem is reduced to the solution of the Fredholm integral equation of the second kind by using the techniques of the Fourier transforms and the finite Hilbert transform. In the case of an uniform opening pressure, the exact expressions for the stress intensity factors in the inner tip and the outer tip of the cracks and the shape of the deformed crack are obtained. Numerical calculations are carried out to study the interaction between the cracks and the effects of the hybrid lamination on the fracture of the hybrid laminate.
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Acta mechanica 13 (1972), S. 117-132 
    ISSN: 1619-6937
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics , Physics
    Description / Table of Contents: Zusammenfassung Eine Störungsrechnung zur Beschreibung der Form des Fernfeldes von Torsionswellen in einem linear-elastischen Stab von elliptischem Querschnitt, mit den Halbachsena undb, wird entwickelt. Die Näherung niedrigster Ordnung, die sich mit bekannten Resultaten vergleichen läßt, führt zu einer in Achsrichtung mit der Geschwindigkeitc = [2ab/(a 2+b 2)](μ/ϱ)1/2 fortlaufenden Welle. Eine Näherung höherer Ordnung, die für große Zeiten gültig ist, wird erhalten; eine Darstellung ihrer Lösung durch ein Airy-Integral zeigt den Dispersionscharakter der Welle.
    Notes: Summary A perturbation method is developed to describe the far-field structure of torsional waves in a linearly elastic rod of elliptical cross section with semi-axes of lengthsa andb. The highest order problem, comparable to what is known, leads to a wave propagating along the axis with a speedc = [2ab/(a 2+b 2)](μ/ϱ)1/2. A higher order approximation is obtained, uniformly valid for large time; a representation of its solution in terms of Airy integral brings out the dispersive nature of the wave.
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  • 8
    ISSN: 0040-4039
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 0142-2421
    Keywords: Chemistry ; Polymer and Materials Science
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Physics
    Notes: Thin (750 Å) plasma-polymerized films of acetylene were deposited onto polished steel substrates in an inductively coupled r.f. reactor. The films were characterized by x-ray photoelectron spectroscopy (XPS), Fourier-transform infrared spectroscopy (FTIR), Auger electron spectroscopy (AES) and secondary ion mass spectrometry (SIMS) immediately after deposition and after aging in the atmosphere. The FTIR spectra of the as-deposited films were characterized by bands related to mono- and disubstituted acetylene groups and by bands related to methyl and methylene groups. During exposure to the atmosphere, bands related to acetylenic groups decreased in intensity while new bands due to carbonyl groups appeared. When XPS spectra were obtained from films that were exposed to the atmosphere, new components assigned to oxidation products were observed in the C 1s spectra that were not observed for as-deposited films, verifying that oxidation had occurred. Numerous peaks related to aromatic structures were observed in positive SIMS spectra of as-deposited films. Results obtained from AES showed that the plasma-polymerized films were continuous and that the oxide on the substrate surface was partially reduced during deposition.Plasma-polymerized acetylene films were excellent primers for rubber-to-steel bonding. Miniature lap joints were prepared by using rubber as an ‘adhesive’ to bond together pairs of polished steel adherends primed with plasma-polymerized acetylene films. The force required to break the as-prepared joints was ∼2000 N for a bonded area of 64 mm2 and failure was 100% cohesive in the rubber. Similar results were obtained for joints prepared using polished brass substrates.Because of the complexity of reactions between rubber and the plasma-polymerized primer, a model ‘rubber’ consisting of a mixture of squalene, zinc oxide, carbon black, sulfur, stearic acid, cobalt naphthenate, N,N-dicyclohexylbenzothiazole sulfenamide and diaryl-p-diphenyleneamine was used to simulate the cross-linking reaction. The results obtained using XPS, SIMS, AES and FTIR showed that sulfur diffused through the primer to form a layer of sulfide at the primer/substrate interface. Zinc and cobalt sulfides and perthiomercaptides, which formed at the interface between squalene and the plasma-polymerized acetylene primer, catalyzed the reaction between squalene and the primer. Cross-links between squalene and the primer were mostly mono-sulfidic, although some evidence for di- and trisulfidic cross-links was observed.
    Additional Material: 17 Ill.
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Journal of elasticity 16 (1986), S. 179-188 
    ISSN: 1573-2681
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics , Physics
    Notes: Abstract The symmetrical problem of the contact between a spherical indenter and a thick transversely isotropic plate is solved using the techniques of the Hankel transform. Solutions are written as the sums of the associated half-space solution and plate thickness effect terms. The normalized normal contact stress and the surface radial stress are obtained and calculated numerically for both composite materials and metallic substances. The example materials give both real and complex characteristic roots. A general method of calculation is described to determine the maximum tensile radial stress and the maximum compressive normal contact stress. The plate thickness effects on the contact stress and on the tensile radial stress are shown to be increasingly important with decreasing thickness. The effects that the material anisotropy has on the magnification of the contact stress and the maximum tensile radial stress are clearly revealed in the numerical results presented. The effects of material anisotropy for the composite are compared to those for the metallic substances.
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