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  • 2000-2004  (2)
  • 1980-1984  (3)
  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science, Ltd
    Allergy 57 (2002), S. 0 
    ISSN: 1398-9995
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Mucopolysaccharidoses ; Sanfilippo D disease ; N-acetylglucosamine-6-sulfate sulfatase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A fourth genetic subtype of the Sanfilippo syndrome due to a deficiency of N-acetylglucosamine-6-sulfate sulfatase which is required for heparan sulfate degradation has recently been described. The clinical findings of two patients with a deficiency of this enzyme are reported here. Differential diagnosis from the other types of the Sanfilippo syndrome cannot be made by clinical criteria, but rests on specific enzyme assays. Since patients of either sex are known and consanguinity was present in one case, autosomal recessive inheritance is most probable.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-203X
    Keywords: Key words Floral organs ; Medicago truncatula ; gfp
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology
    Notes: Abstract  A high frequency of embryogenesis and transformation from all parts of flowers of two lines of Medicago truncatula R-108–1 and Jemalong J5 were obtained. Using this flower system, we obtained transgenic plants expressing promoter-uidA gene fusions as well as the gfp living cell color reporter gene. Moreover, this method allows us to save time and to use a smaller greenhouse surface for the culture of donor plants. Southern hybridization showed that the internal gfp fragment had the expected size and the number of T-DNA copies integrated in the plant genome varied between one and three. These data suggest that the presence of the GFP protein has no toxic effects, since no rearrangement of the gfp reporter gene was detected in the regenerated plants.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Journal of inherited metabolic disease 5 (1982), S. 218-224 
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A female patient of Italian, non-Jewish, descent, 22 years of age, with cloudy corneae, capsular lens opacities and severe and progressive mental and motor deterioration is described. Ultrastructural examination of a skin biopsy sample showed storage of membranous cytoplasmic bodies in Schwann cells, vessel walls, fibroblasts, smooth muscle fibres and sweat glands, and the presence of some electron-lucent vacuoles, filled with fibrillo-granular material, in sweat glands. Biochemical analysis of cultured fibroblasts (from skin explant) showed a complete deficiency of the sialidase acting on gangliosides, while the sialidase acting on sialyllactose and MU-NeuAc, and several lysomal hydrolases were normal. The urine sediment analysis showed accumulation of all phospholipid species, of several glycolipids and of gangliosides, especially of the polysialylated species. We conclude that the patient under examination is affected by Mucolipidosis IV and the term ‘sialolipidosis’ is suggested for this inborn disorder.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Boundary layer meteorology 28 (1984), S. 51-77 
    ISSN: 1573-1472
    Source: Springer Online Journal Archives 1860-2000
    Topics: Geosciences , Physics
    Notes: Abstract The ECLATS experiment was conducted in order to investigate the influence of radiative processes on the dynamics of the atmospheric boundary layer during its diurnal evolution. This experiment was carried out over Niger, near Niamey, by measuring continuously the energy balance at ground level and by using an instrumented aircraft for turbulence, radiative fluxes and aerosol measurements in the boundary layer during dusty conditions (‘brumes sèches’). This paper is restricted to an analysis of the turbulent structure in the homogeneous and stationary convective boundary layer. The turbulence moments for kinetic energy and the spectral characteristics of the vertical velocity are discussed. These results are compared with a set of data obtained for clear convective boundary layers. The differences observed are quite important and seem, at least in part, due to radiative processes (infrared radiative divergence in the surface layer and absorption of solar radiation in the boundary layer).
    Type of Medium: Electronic Resource
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