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  • 1
    Electronic Resource
    Electronic Resource
    The clinical, genetic and dystrophin characteristics of Becker muscular dystrophy (1993)
    Springer
    Journal of neurology 240 (1993), S. 105-112 
    Details
    ISSN: 1432-1459
    Keywords: Muscular dystrophy ; Phenotype-genotype correlation ; Dystrophin ; Gene deletions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We have correlated a detailed clinical assessment of 67 patients with proven Becker muscular dystrophy with the results from genetic and protein analyses. There was an overall deletion frequency of 80%, rising to 92.6% in the large group of patients defined on clinical grounds as being of “typically” mild severity. The deletions in this group were all clustered in the region of the gene between exons 45 and 59; the most common deletion was of exons 45–47 and all but one started at exon 45. No similar deletions were seen in the patients with more severe disease, in whom the diverse genetic defects included a duplication and a very large deletion. Dystrophin patterns in the “typical” group were also very characteristic, and in both groups were as predicted from the genetic defect, the size of deletions being inversely proportional to the size of the protein produced.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00858726
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    The distribution of desmin and titin in normal and dystrophic human muscle (1992)
    Springer
    Acta neuropathologica 83 (1992), S. 158-169 
    Details
    ISSN: 1432-0533
    Keywords: Skeletal muscle ; Muscular dystrophy ; Titin ; Desmin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We have used monoclonal antibodies to desmin and titin, and a combination of immunofluoescence and immunogold labelling to study the disposition of these two proteins in normal human muscle fibres and in fibres at various stages of degeneration in dystrophic muscle. The normal pattern of desmin labelling, in particular the subsarcolemmal labelling, became disrupted at an early stage of fibre breakdown. There was a change from a transverse to a longitudinal orientation of the labelled intermediate filaments as the myofibrils sheared relative to one another. Thus, while it is probable that the desmin filaments are able to play a role in the mechanical integration of the myofibrils in healthy muscle, our results suggest that they cannot withstand the excessive forces generated by the hypercontraction and stretching of dystrophic muscle. However, small accumulations of desmin persisted between the damaged myofibrils until necrosis reached an advanced stage. In general, the degradation of titin appeared to occur before the degradation of desmin, and at the ultrastructural level, labelling with antibodies to epitopes from parts of the titin molecule close to the A-I-band junction was lost before labelling with an antibody to an epitope in the A-band. This suggests that different regions of the titin molecule break down at different stages in the breakdown of the fibre. We propose that lysis of titin in the I-band may underlie ‘slippage’, an abnormality often seen in dystrophic muscle, in which the A-band slips to one pole of the sarcomere such that it abuts onto the Z-line. Breakdown of the A-band section of titin may facilitate the disassembly of the A-filaments.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00308475
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Kinetic limitations to surface segregation during MBE growth of III–V compounds: Sn in GaAs (1984)
    Springer
    Applied physics 33 (1984), S. 87-92 
    Details
    ISSN: 1432-0630
    Keywords: 73.60
    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 incorporation of Sn as a dopant in GaAs has been studied in the temperature range of 500°–650° C, over a wide range of Ga and As fluxes, the latter being incident as either As4 or As2 molecules. The results are explained in terms of a surface segregation model in which the behaviour at high growth temperatures (above ∼600 °C) approaches thermal equilibrium, but growth at lower temperatures involves a kinetic limitation to the segregation process.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00617613
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Nucleation effects during MBE growth of Sn-Doped GaAs (1982)
    Springer
    Applied physics 28 (1982), S. 63-71 
    Details
    ISSN: 1432-0630
    Keywords: 73.60
    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 morphology, structure and composition of Sn films on GaAs substrates have been investigated in relation to the predeposition technique used with Sn doping of MBE-grown GaAs films. It has been shown that 3-D aggregates of Sn are formed and a similar morphology has been identified in the Sn which accumulates at the surface of all Sn-doped GaAs films. The way in which this relates to donor incorporation is considered and a simple model of incorporation kinetics proposed which is shown to be consistent with observation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00617784
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    Paper (German National Licenses)
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  • 5
    Electronic Resource
    Electronic Resource
    Silicon doping of MBE-grown GaAs films (1983)
    Springer
    Applied physics 32 (1983), S. 195-200 
    Details
    ISSN: 1432-0630
    Keywords: 73.60
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics , Physics
    Notes: Abstract Two concentration ranges of silicon doping in MBE-grown GaAs films have been investigated in some detail. In lightly doped films, with a free-electron concentration of ≈1016cm−3, low-temperature photoluminescence spectra have been analysed to develop a model to account for spectral features previously attributed to Ge and Si acceptor levels. In heavily doped films, a maximum free-electron concentration of ≈7×1018 cm−3 has been obtained, which is only rather weakly dependent on growth conditions and the nature of the arsenic species (As2 or As4). Transmission electron microscopy has shown that no significant precipitation effects occur when higher Si fluxes are used but there is evidence for autocompensation. The maximum PL intensity (300 K) is found at a lower free electron concentration then with Sn-doped films, and is more sharply peaked, but there is no evidence for an anomalous Moss-Burstein shift.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00820260
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    Paper (German National Licenses)
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