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  • 1
    Electronic Resource
    Electronic Resource
    Stereological and morphometric analysis of dermal fibroblasts before and after bone marrow transplantation in a case of mucopolysaccharidosis I Scheie phenotype (1993)
    Springer
    Acta neuropathologica 86 (1993), S. 21-28 
    Details
    ISSN: 1432-0533
    Keywords: Mucopolysaccharidosis I Scheie phenotype ; Bone marrow transplantation ; Fibroblasts ; Stereologic analysis ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Bone marrow transplantation (BMT) has been used therapeutically in several types of mucopoly-saccharidoses (MPS) and other inherited metabolic disorders. Fibroblasts are severely affected in MPS due to the intralysosomal accumulation of glycosaminoglycans. We report a stereological and morphometric study at light and electron microscopy levels of dermal fibroblasts before and 21 months after BMT in a young girl with MPS I Scheie phenotype (MPS I-S). Dermal fibroblasts showed remarkable morphological changes although their density, expressed as number of fibroblasts per unit volume of dermis (number density), was not modified in the post-BMT samples as compared to pre-BMT ones. Stereological and morphometric parameters referring to cell characteristics of post-BMT fibroblasts (nuclear and cell surface densities, and both nucleus/cell and cell/nucleus volume densities) showed significant differences when compared with pre-BMT fibroblasts, and non-significant differences regarding control cells. On the other hand, quantitative parameters of the lysosomal compartment from post-BMT fibroblasts showed intermediate values between pre-BMT and control fibroblasts. These results, at cellular level, are in agreement with previous biochemical and clinical results, and clearly showed a progressive course to a non-pathological state. All parameters estimated may be considered useful tools in evaluating the success of BMT. These parameters provide quantitative data which can be statistically compared, showing the changes due to the reduction of storage material after BMT. Cell/nucleus volume density is especially interesting since not only is it easy to estimate, even by automatic procedures, but it could also constitute a numerical expression of skin anatomopathological analyses performed post-BMT.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00454894
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  • 2
    Electronic Resource
    Electronic Resource
    Bone marrow transplant in a case of mucopolysaccharidosis I Scheie phenotype: skin ultrastructure before and after transplantation (1991)
    Springer
    Acta neuropathologica 82 (1991), S. 33-38 
    Details
    ISSN: 1432-0533
    Keywords: MPS I-S ; Skin biopsy ; Ultrastructure ; Bone marrow transplant
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An 11-year-old girl with mucopolysaccharidosis I Scheie phenotype (MPS I-S) received a bone marrow transplant (BMT) from her heterozygous HLA-identical LMC-non-reactive mother. Multidisciplinary studies were carried out and results evaluated 21 months after transplantation. Herein we report the ultrastructural findings pre-and post-BMT in skin. Multidisciplinary studies are commonly used to evaluate the benefits of metabolic correction following BMT in some MPS and other inherited metabolic disorders, and changes in morphology have been described in liver and few other tissues. In this case, we elected skin, since connective tissue is universally involved in MPS and is safely and easily obtainable. Comparison of skin biopsy specimens taken before and after BMT showed a considerable change in dermal fibroblast morphology, with marked reduction in cell size and the number and size of abnormal lysosomes, thus indicating the clearance of storage. Our results demonstrate that dermal cells respond to enzyme replacement therapy in MPS I-S, with the clearance of glycosaminoglycan lysosomal accumulation in connective tissue fibroblasts, which had near-normal morphology 21 months after BMT. Therefore, the practice of skin biopsy after BMT in MPS and other metabolic disorders in which dermal cells are involved should be encouraged.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00310920
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Ultrastructural studies of the myenteric plexus and smooth muscle in organotypic cultures of the guinea-pig small intestine (1995)
    Springer
    Cell & tissue research 280 (1995), S. 627-637 
    Details
    ISSN: 1432-0878
    Keywords: Key words: Myenteric plexus ; Smooth muscle ; Organotypic culture ; Ultrastructure ; Intestine ; small ; Guinea-pig
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract. External muscle and myenteric plexus from the small intestine of adult guinea-pigs were maintained in vitro for 3 or 6 days. Myenteric neurons and smooth muscle cells from such organotypic cultures were examined at the electron-microscopic level. An intact basal lamina was found around the myenteric ganglia and internodal strands. Neuronal membranes, nuclei and subcellular organelles appeared to be well preserved in cultured tissues and ribosomes were abundant. Dogiel type-II neurons were distinguishable by their elongated electron-dense mitochondria, numerous lysosomes and high densities of ribosomes. Vesiculated nerve profiles contained combinations of differently shaped vesicles. Synaptic membrane specializations were found between vesiculated nerve profiles and nerve processes and cell bodies. The majority of nerve fibres were well preserved in the myenteric ganglia, in internodal strands and in bundles running between circular muscle cells. No detectable changes were found in the ultrastructure of the somata and processes of glial cells. Longitudinal and circular muscle cells from cultured tissue had clearly defined membranes with some close associations with neighbouring muscle cells. Caveolae occurred in rows that ran parallel to the long axis of the muscle cells. These results indicate that the ultrastructural features of enteric neurons and smooth muscle of the guinea-pig small intestine are well preserved in organotypic culture.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00318365
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Structure of the tertiary component of the myenteric plexus in the guinea-pig small intestine (1993)
    Springer
    Cell & tissue research 272 (1993), S. 509-516 
    Details
    ISSN: 1432-0878
    Keywords: Myenteric plexus ; Enteric nervous system ; Intestine, small ; Ultrastructure ; Innervation, of intestinal muscle ; Guinea-pig
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract The tertiary component of the myenteric plexus consists of interlacing fine nerve fibre bundles that run between its principal ganglia and connecting nerve strands. It was revealed by zinc iodide-osmium impregnation and substance P immunohistochemistry at the light-microscope level. The plexus was situated against the inner face of the longitudinal muscle and was present along the length of the small intestine at a density that did not vary markedly from proximal to distal. Nerve bundles did not appear to be present in the longitudinal muscle as judged by light microscopy, although numberous fibre bundles were encountered within the circular muscle layer. At the ultrastructural level, nerve fibre bundles of the tertiary plexus were found in grooves formed by the innermost layer of longitudinal smooth muscle cells. In the distal parts of the small intestine, some of these nerve fibre bundles occasionally penetrated the longitudinal muscle coat. Vesiculated profiles in nerve fibre bundles of the tertiary plexus contained variable proportions of small clear and large granular vesicles; they often approached to within 50–200 nm of the longitudinal smooth muscle cells. Fibroblast-like cells lay between strands of the tertiary plexus and the circular muscle but were never intercalated between nerve fibre varicosities and the longitudinal muscle. These anatomical relationships are consistent with the tertiary plexus being the major site of neurotransmission to the longitudinal muscle of the guinea-pig small intestine.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00318557
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    Paper (German National Licenses)
    Fulltext
  • 5
    Electronic Resource
    Electronic Resource
    Ultrastructural studies of the myenteric plexus and smooth muscle in organotypic cultures of the guinea-pig small intestine (1995)
    Springer
    Cell & tissue research 280 (1995), S. 627-637 
    Details
    ISSN: 1432-0878
    Keywords: Myenteric plexus ; Smooth muscle ; Organotypic culture ; Ultrastructure ; Intestine, small ; Guinea-pig
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract External muscle and myenteric plexus from the small intestine of adult guinea-pigs were maintained in vitro for 3 or 6 days. Myenteric neurons and smooth muscle cells from such organotypic cultures were examined at the electron-microscopic level. An intact basal lamina was found around the myenteric ganglia and internodal strands. Neuronal membranes, nuclei and subcellular organelles appeared to be well preserved in cultured tissues and ribosomes were abundant. Dogiel type-II neurons were distinguishable by their elongated electron-dense mitochondria, numerous lysosomes and high densities of ribosomes. Vesiculated nerve profiles contained combinations of differently shaped vesicles. Synaptic membrane specializations were found between vesiculated nerve profiles and nerve processes and cell bodies. The majority of nerve fibres were well preserved in the myenteric ganglia, in internodal strands and in bundles running between circular muscle cells. No detectable changes were found in the ultrastructure of the somata and processes of glial cells. Longitudinal and circular muscle cells from cultured tissue had clearly defined membranes with some close associations with neighbouring muscle cells. Caveolae occurred in rows that ran parallel to the long axis of the muscle cells. These results indicate that the ultrastructural features of enteric neurons and smooth muscle of the guinea-pig small intestine are well preserved in organotypic culture.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00318365
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
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