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  • 1
    ISSN: 1432-0533
    Keywords: Dystrophin ; Immunoblot analysis ; Neuromuscular junction ; Myotendinous junction ; Dystrophin-related protein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary To determine whether or not and how dystrophin exists in neuromuscular junctions (NMJs) and myotendinous junctions (MTJs), we studied the mid-belly and peripheral portions of control and mdx muscles, immunohistochemically and immunoelectrophoretically, using six kinds of polyclonal antibodies, and an antibody against a dystrophin-related protein (DRP). In controls these regions and the polar region of intrafusal muscle fibers showed a rather clearer immunohistochemical dystrophin reaction than those of extrafusal muscle fibers with all antibodies used. In the muscles of mdx mice NMJs only showed a positive dystrophin reaction with the c-terminal antibody, that is, no reaction with the other five antibodies, and MTJs in mdx showed a positive reaction with the c-terminal antibody and a faint to negative reaction with the other five antibodies. In biopsied human muscles NMJs and MTJs also showed a clear reaction with all ten antibodies, i.e., six polyclonal and four monoclonal ones. Although an immunohistochemical DRP reaction was clearly seen at NMJs, only a faint or no reaction was seen on MTJs and on intrafusal muscle fibers in both mouse and human materials. Western blot analysis of control mouse muscle for dystrophin showed a clearer band for the peripheral portion, which contains many MTJs, than for the mid-belly portion. These data suggest that dystrophin really exists on MTJs, and that dystrophin and DRP exist on NMJs in mouse and human muscles.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Acridine Orange ; RNA ; Regeneration ; Denervation ; Fluorescent microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The Acridine Orange (AO) stain for muscle biopsies is particularly useful to identify regenerating and ongoing hypertrophic muscle fibers under fluorescent microscopy. This method was applied to muscle biopsies from 65 patients who suffered from various childhood neuromuscular disorders. While normal fibers showed dull green cytoplasm with small green-yellow nuclei, striking fluorescent fibers were observed in eight cases of congenital muscular dystrophy (CMD) and 12 cases of Duchenne muscular dystrophy (DMD); these fibers were characterized as follows: (1) small fibers with big oval or spherical nucleic which fluoresced strongly with a bright orange color; (2) fibers of various sizes and different degrees of orange fluorescence; and (3) opaque fibers with bright yellow cytoplasm. The small diameter fibers in Werdnig-Hoffmann (WH) disease, nemaline myopathy, and congenital fiber type dysproportion failed to show apparent AO-RNA fluorescence. Although all the atrophic fibers in Kugelberg-Welander (KW) disease showed a vague orange fluorescent color, this was obviously different from that of regenerating fibers seen in CMD and DMD. In addition to these findings, the hypertrophic fibers in a case of unclassified myopathy also showed moderate organe fluorescence around the entire periphery of the cytoplasm.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 69 (1986), S. 117-123 
    ISSN: 1432-0533
    Keywords: Skeletal muscle ; Intramuscular nerves ; Infantile Neuroaxonal Dystrophy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Biopsies of the biceps muscle and sural nerve were taken from a girl aged 2 years with infantile neuroaxonal dystrophy (INAD). In addition to the typical axonal spheroid bodies in a number of the i. m. nerve fibers, the neuromuscular junctions (NMJs) and motor nerve endings also contained axonal swellings. The sural nerve, except for three dystrophic fibers, was almost completely normal. A teased nerve preparation showed four additional abnormal fibers with focal axonal enlargement similar to those in giant axonal neuropathy (GAN). These results suggest that a biceps muscle biopsy may be more useful than a sural nerve biopsy for the diagnosis of INAD, because the muscle contains abnormal peripheral nerves and NMJs in high frequency.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 75 (1988), S. 308-312 
    ISSN: 1432-0533
    Keywords: Duchenne muscular dystrophy ; Opaque fibers ; Necrotic fibers ; Regenerative fibers
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We studied the incidence of opaque, necrotic, and regenerative muscle fibers (OFs, NFs, RFs) in muscle biopsies from 21 patients, aged 8 months to 24 years, with Duchenne muscular dystrophy (DMD). OFs varied from case to case with an average rate of 8.3%. NFs and RFs were high in younger patients and decreased with age. The incidence of OFs, NFs, and RFS correlated to patient age with these coefficients:r=−0.38, −0.91, and −0.86. Except for OFs, all correlations were significant (p〈0.001). There was no significant correlation between the incidence of OFs and NFs in each case of DMD. Considering the results of previous biochemical analysis of single OFs, we suggest that OFs may comprise two types of muscle fibers: (1) pathological muscle fibers to be regarded as precursors of NFs, and (2) muscle fibers undergoing artificial changes.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-0533
    Keywords: Serotonin ; Dystrophin ; Duchenne muscular dystrophy ; Type IV collagen ; Laminin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Degenerating and regenerating muscle fibers, in serotonin-induced myopathy (SM) of rats, were investigated histochemically, immunohistochemically and electron microscopically with polyclonal antibodies against dystrophin, type IV collagen and laminin. The myopathy produced was characterized by grouping of degenerating and regenerating muscle fibers, and degeneration of capillary endothelial cells. Dystrophin disappeared in an early stage of muscle degeneration and reappeared in an early stage of regeneration. On the other hand, type IV collagen and laminin were well preserved throughout the degeneration and regeneration processes, even on the shrunk and wrinkled basement membrane of empty muscle fibers after phagocytosis. Muscle fiber regeneration was completed within each tube of the preserved basement membrane through the fusion of myoblasts derived from satellite cells of single necrotic fibers, myotubes already being visible on the 1st or 2nd day of regeneration on light microscopy. These small regenerating myotubes did not fuse with each other at all. The findings in the present experimental SM study are compatible with those in Duchenne muscular dystrophy, especially at the preclinical stage.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Pediatric allergy and immunology 1 (1990), S. 0 
    ISSN: 1399-3038
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Expression of low-affinity receptors for IgE (FcɛRII) on T and B cells was examined with monoclonal antibodies to cell surface antigens of FcɛRII (CD23), T cells (CD3) or B cells (CD 19) by two-dimensional analysis under laser flow cytometry system. The majority of cells bearing FcɛRII were B cells; however, a small proportion of T cells (1. 6%) bore FcɛRII in children with atopic asthma and elevated serum level of IgE. The occurrence of FcɛRII positive B cells was also increased in children with atopic asthma compared with non-atopic controls (p 〈 0.001). The numbers of T and B cells hearing Fc receptors for IgG (FcɛR) did not differ between atopic and non-atopic children. T cells were isolated from venous blood by rosetting with sheep erythrocytes, and then cultured in vitro with phytohemagglutinin plus phorbol-myristate-acetate. The amount of IgE-binding factors (IgE-BF), a soluble form of FcɛRII, was determined by means of enzyme-linked immunosorbent assay. The level of IgE-BF was increased in cultures from children with a atopic asthma compared with non-atopic controls (p 〈 0.01). The possible involvements of FcɛRII on T cells and their soluble products, IgE-BF, in the pathogenesis of atopic asthma is discussed.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Copenhagen : Munksgaard International Publishers
    Allergy 56 (2001), S. 0 
    ISSN: 1398-9995
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Allergy 51 (1996), S. 0 
    ISSN: 1398-9995
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Annals of the New York Academy of Sciences 320 (1979), S. 0 
    ISSN: 1749-6632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Natural Sciences in General
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Hereditary dentatorubral–pallidoluysian atrophy (DRPLA) is an autosomal dominant neurologic disorder characterized by variable combinations of myoclonus, epilepsy, cerebellar ataxia, choreoathetosis and dementia. By specifically searching published brain cDNA sequences for the presence of CAG ...
    Type of Medium: Electronic Resource
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