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  • Biochemistry  (1)
  • GM1-gangliosidosis  (1)
  • jet impingement  (1)
  • 1
    Electronic Resource
    Electronic Resource
    Retarded bone formation in GM1-gangliosidosis: a study of the infantile form and comparison with two canine models (1995)
    Springer
    Virchows Archiv 426 (1995), S. 141-148 
    Details
    ISSN: 1432-2307
    Keywords: Retarded bone formation ; GM1-gangliosidosis ; Canine model
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The development of skeletal lesions in two canine models of GM1-gangliosidosis, English springer spaniels and Portuguese water dogs, has been studied and compared to osseous abnormalities in a child with the infantile form of the disease. In the canine models, skeletal dysplasia was progressive. Lesions were noted at 2 months of age and characterized by retarded endochondral ossification and osteoporosis. Older puppies had focal cartilage necrosis within lumbar vertebral epiphyses. At the cellular level, lesions were characterized by chondrocytic hypertrohy and lysosomal accumulation of storage compounds. Our studies illustrate that the skeletal lesions in both canine models are similar to those in a child with GM1-gangliosidosis. Furthermore, we proposed that the abnormal storage of partially degraded compounds in affected chondrocytes might explain, at least in part, the retarded bone formation noted in patients with GM1-gangliosidosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00192635
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    Paper (German National Licenses)
    Fulltext
  • 2
    Electronic Resource
    Electronic Resource
    Adult-onset lysosomal storage disease in a Schipperke dog: clinical, morphological and biochemical studies (1993)
    Springer
    Acta neuropathologica 86 (1993), S. 306-312 
    Details
    ISSN: 1432-0533
    Keywords: Canine galactosialidosis ; Morphology ; Biochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An adult-onset lysosomal storage disorder was diagnosed in a 5-year-old Schipperke dog with progressive cerebellar and central vestibular signs. It was characterized by cerebellar atrophy with extensive loss of Purkinje and granular cells, and hydrocephalus. Enlarged and vacuolated neurons were observed in spinal cord and brain; pancreatic centrolobular and islet cells were also vacuolated. Ultrastructurally, enlarged secondary lysosomes laden with lamellated membrane structures were present in neurons and empty enlarged vacuoles were found in pancreatic centroacinar, ductal, and islet cells. On frozen sections neurons stained with Ricinus communis agglutinin-I and wheat germ agglutinin. On paraffin sections neurons stained with luxol fast blue, periodic acid-Schiff, Concanavalia ensiformis agglutinin, and were autofluorescent. These findings indicate an accumulation of glycolipids containing terminal β-galactosyl and α-sialyl residues, and N-linked oligosaccharides. Tissue activity of lysosomal β-galactosidase was 50% of normal and the activity of β-hexosaminidase was elevated. Brain lipid-bound sialic acid was twice normal, with a small increase of GM1-ganglioside, but there was a significant elevation of GM2 (GD2) and GM3 (GD3). In addition, significant elevations of sialylated and non-sialylated oligosaccharides were noted. These clinical, biochemical and pathological findings are similar to those observed in human patients with adult-onset galactosialidosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00304147
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    Paper (German National Licenses)
    Fulltext
  • 3
    Electronic Resource
    Electronic Resource
    COMPUTATIONAL STUDIES OF IMPINGING JETS USING K-∊ TURBULENCE MODELS (1996)
    Chichester : Wiley-Blackwell
    International Journal for Numerical Methods in Fluids 22 (1996), S. 799-810 
    Details
    ISSN: 0271-2091
    Keywords: jets ; jet impingement ; turbulence ; k-ε model ; finite volume method ; Engineering ; Engineering General
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Notes: This paper reports numerical modelling of impinging jet flows using Rodi and Malin corrections to the k-ε turbulence model, carried out using the PHOENICS finite volume code. Axisymmetric calculations were performed on single round free jets and impinging jets and the effects of pressure ratio, height and nozzle exit velocity profile were investigated numerically. It was found that both the Rodi and Malin corrections tend to improve the prediction of the hydrodynamic field of free and impinging jets but still leave significant errors in the predicted wall jet growth. These numerical experiments suggest that conditions before impingement significantly affect radial wall jet development, primarily by changing the wall jet's initial thickness.
    Additional Material: 11 Ill.
    Type of Medium: Electronic Resource
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    Paper (German National Licenses)
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