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  • 1
    ISSN: 1432-0533
    Keywords: Key words Cytoskeletal proteins ; Immunohistochemistry ; Myofibrillar myopathy ; Hereditary myopathy ; Holstein-Friesian cattle
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We have investigated the expression, using immunohistochemical and Western blot methods, of some cytoskeletal proteins including desmin, vimentin, actin, α-actinin, and ubiquitin in hereditary myopathy of the diaphragmatic muscles in Holstein-Friesian cattle (the histochemical and electron microscopical aspects have been previously reported). Immunohistochemically, the expression of desmin was observed strongly in the subsarcolemmal regions, but was lacking or faint in the area corresponding to the core-like structures. Vimentin showed almost the same localization as desmin, but no activity could be observed in the core-like structures. In addition, the core-like structures showed strong immunoreactivity for actin and ubiquitin, but no immunoreactivity for α-actinin. F-actin stained with phalloidin-tetramethyl-rhodamine was strongly positive in irregular spots that corresponded to the core-like structures, but was negative for desmin-positive regions. Western blot analysis of the diseased muscles revealed a significant increase in the amount of desmin and vimentin immunoreactivities and similar amounts of actin and α-actinin compared with the control muscles. Two-dimensional electrophoresis revealed no isoforms of desmin, suggesting the absence of abnormal phosphorylated forms of desmin. Since the co-localization of desmin and vimentin and the absence of phosphorylated desmin suggest that the overexpression of desmin may be reflected in the reactive change or regenerating process, the present myopathy should be regarded as an entity separate from desmin-storage myopathy or desmin-related myopathies. We also discuss the possibility that the present myopathy could be considered as myofibrillar myopathy, a recently proposed nosological entity.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Key words Myocardium ; Intracytoplasmic inclusion ; Hereditary myopathy ; Diaphragmatic muscles ; Cattle
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In hereditary myopathy of the diaphragmatic muscles in Holstein-Friesian cattle, the largest number of acidophilic intracytoplasmic inclusions was found in the myocardium. These inclusions, which were oval and measured 12–15 μm in the transverse sections, were characterized by a dense, amorphous zone, and a relatively hyalinized sarcous substance in paraffin-embedded hematoxylin and eosin (H & E)-stained sections. Histochemically, each inclusion was stained intense red and dark green with H & E and Gomori’s trichrome, respectively. NADH-TR activity was absent. The region surrounding the inclusions was less acidophilic with H & E, and showed an increased activity with NADH-TR. The inclusions showed no immunoreactivity for desmin, vimentin, actin or α-actinin, while strong desmin immunoreactivity was observed in the region surrounding the inclusion. Some inclusions showed strong immunoreactivity for ubiquitin, but others reacted only faintly. Ultrastructurally, the inclusion had a dense core composed of myofibrillar aggregations. The periphery of this dense core was surrounded by thin or intermediate-sized filaments, which corresponded to the desmin-positive area. This alteration was sometimes found to be continuous with the Z disk, which showed streaming or disintegration or with the desmosome of the intercalated disk. We discuss here the similarity between this specific inclusion and the other alternative organelles that have been reported previously in cardiomyopathy or in cardiac lesions accociated with various myopathies.
    Type of Medium: Electronic Resource
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