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  • 1
    Electronic Resource
    Electronic Resource
    Localization of a gene for Fukuyama type congenital muscular dystrophy to chromosome 9q31–33 (1993)
    [s.l.] : Nature Publishing Group
    Nature genetics 5 (1993), S. 283-286 
    Details
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Fukuyama type congenital muscular dystrophy (FCMD) is an autosomal recessive severe muscular dystrophy associated with an anomaly of the brain. Twenty–one FCMD families, 13 of them with consanguineous marriages, were analysed by genetic linkage analyses with polymorphic microsatellite markers ...
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1038/ng1193-283
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  • 2
    Electronic Resource
    Electronic Resource
    Progression in nemaline myopathy (1989)
    Springer
    Acta neuropathologica 78 (1989), S. 484-491 
    Details
    ISSN: 1432-0533
    Keywords: Nemaline myopathy ; Lysosomal enzymes ; Acid phosphatase ; Cathepsins ; Myofibrillar degeneration
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Four of seven patients with nemaline myopathy had severe, rapidly progressing symptoms. These four showed an increase in acid phosphatase activity in muscle fibers demonstrated by histochemistry and cathepsin B&L activity by biochemical measurement. On electron microscopy, nemaline bodies, occasionally disorganized myofibrils and autophagic vacuoles containing sarcoplasmic debris and glycogen particles were seen. Focal myofibrillar degeneration, through an unknown pathogenetic mechanism, induces an increase in lysosomal enzymes in the skeletal muscles which may be closely correlated with a rapid aggravation of muscle weakness in nemaline myopathy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687709
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  • 3
    Electronic Resource
    Electronic Resource
    Initiation of satellite cell replication in bupivacaine-induced myonecrosis (1994)
    Springer
    Acta neuropathologica 88 (1994), S. 252-257 
    Details
    ISSN: 1432-0533
    Keywords: Satellite cell ; Satellite cell replication ; Regeneration ; Bupivacaine ; Bromodeoxyuridine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To determine how and when the satellite cells are stimulated to replicate in muscle regeneration, the rat soleus muscle was examined chronologically after bupivacaine-induced myonecrosis. Bromodeoxyuridine and desmin-positive mononuclear cells, indicating the start of satellite cell replication, were seen 25 h after bupivacaine treatment when macrophages had already invaded the sarcoplasm of necrotic fiber. These findings suggest that muscle regeneration starts as early as the time at which macrophages begin to scavenge necrotic material. Proliferating myoblasts increased in number, reaching a maximum at 49 h after myonecrosis, and decreased in number 3 days after the myoblasts fused with each other to form myotubes. The satellite cell proliferation after bupivacaine-induced myonecrosis began at almost the same time as in crush injury, and earlier than after muscle transplantation using whole intact or minced muscle fragments. The earlier begining and more rapid regenerating process probably resulted from the preservation of intact satellite cells, blood vessels and peripheral nerves in the bupivacaine-induced myonecrosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00293401
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  • 4
    Electronic Resource
    Electronic Resource
    Initiation of satellite cell replication in bupivacaine-induced myonecrosis (1994)
    Springer
    Acta neuropathologica 88 (1994), S. 252-257 
    Details
    ISSN: 1432-0533
    Keywords: Key words Satellite cell ; Satellite cell replication ; Regeneration ; Bupivacaine ; Bromodeoxyuridine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To determine how and when the satellite cells are stimulated to replicate in muscle regeneration, the rat soleus muscle was examined chronologically after bupivacaine-induced myonecrosis. Bromodeoxyuridine and desmin-positive mononuclear cells, indicating the start of satellite cell replication, were seen 25 h after bupivacaine treatment when macrophages had already invaded the sarcoplasm of necrotic fiber. These findings suggest that muscle regeneration starts as early as the time at which macrophages begin to scavenge necrotic material. Proliferating myoblasts increased in number, reaching a maximum at 49 h after myonecrosis, and decreased in number 3 days after the myoblasts fused with each other to form myotubes. The satellite cell proliferation after bupivacaine-induced myonecrosis began at almost the same time as in crush injury, and earlier than after muscle transplantation using whole intact or minced muscle fragments. The earlier begining and more rapid regenerating process probably resulted from the preservation of intact satellite cells, blood vessels and peripheral nerves in the bupivacaine-induced myonecrosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00293401
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  • 5
    Electronic Resource
    Electronic Resource
    Comparison of behavior in muscle fiber regeneration after bupivacaine hydrochloride- and acid anhydride-induced myonecrosis (1992)
    Springer
    Acta neuropathologica 83 (1992), S. 584-589 
    Details
    ISSN: 1432-0533
    Keywords: Muscle necrosis ; Regeneration ; Fibrosis ; Bupivacaine ; Acid anhydride
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We compared the morphologic characteristics of muscle fiber necrosis and subsequent regeneration after injury induced by intramuscular injections of bupivacaine hydrochloride (BPVC) and a variety of solutions at acid and alkaline pH (acetic anhydride, citric acid buffer, and sodium carbonate buffer). After BPVC injection the necrotic muscle fibers were rapidly invaded by phagocytic cells, followed by active regeneration and very little fibrous scar formation. The regenerating muscle fibers increased rapidly in size and attained complete fiber type differentiation and regained their initial fiber diameter within 1 month. Both alkaline and acid solutions induced muscle fiber necrosis followed by regeneration. Fiber necrosis induced by alkaline buffers and acetic anhydride solutions above pH 5.0 produced changes quite similar to that induced by BPVC. However, injection with 0.1 M acetic anhydride at pH below 4.0 resulted in coagulative necrosis of the injured muscle with very little phagocytic infiltration with poor regenerative activity and dense fibrous tissue scarring. Thus, pH 4.0 appears to be the critical pH determining the type of muscle injury and subsequent poor phagocytic and regenerative activities. This model of acidic acetic anhydride injury may lead to the identification of factors which interfere with regeneration and cause fibrous tissue scarring in human muscular dystrophy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00299406
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  • 6
    Electronic Resource
    Electronic Resource
    Acid maltase deficiency in the Japanese quail; early morphological event in skeletal muscle (1987)
    Springer
    Acta neuropathologica 73 (1987), S. 32-37 
    Details
    ISSN: 1432-0533
    Keywords: Acid maltase deficiency ; Japanese quail ; Early morphological change ; Membrane-bound glycogen
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The skeletal muscle of Japanese quails with acid maltase deficiency (AMD) was studied morphologically at various developmental stages, from the 16th embryonal day up to 3 months after hatching. Membrane-bound glycogen particles began to appear in the affected skeletal muscle at the 16th embryonal day. In normal embryonic muscles, a certain amount of free glycogen particles was observed but they were not membrane-bound. Therefore, this is the earliest morphological event in the muscle of Japanese quails with AMD. In muscle at 3 weeks after hatching, the initial focal degeneration of myofibrils was recognizable but it was not associated with autophagic vacuoles. Quails with AMD developed muscle weakness and difficulty in lifting their wings at about 3 months after hatching: then numerous autophagic vacuoles were present. The formation of large autophagic vacuoles followed by fiber loss and fatty replacement seemed ot contribute to the progressive muscle weakness. The study of Japanese quail with AMD will greatly facilitate the elucidation of the pathogenetic mechanism and is also a useful model for therapeutic trials in human AMD.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00695499
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  • 7
    Electronic Resource
    Electronic Resource
    Shaking rat Kawasaki (SRK): a new neurological mutant rat in the Wistar strain (1988)
    Springer
    Acta neuropathologica 76 (1988), S. 366-372 
    Details
    ISSN: 1432-0533
    Keywords: Cortical dysplasia ; Neuronal migration disorder ; Neurological mutant ; Shaking rat ; Kawasaki (SRK) ; Wistar rat
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Shaking rat Kawasaki (SRK), a newly discovered neurological mutant rat in the Wistar strain, is described. The abnormalities of SRK rats are transmitted as an autosomal recessive trait. The neurological signs are shaking of the body and an ataxic-paretic gait from day 10 postnatal. The affected rats survive for about 1 month. Macroscopically, the cerebellum is small and frequently the vermis and paraflocculus lacking. The most conspicuous histological finding in the central nervous system is malposition of the neurons in the cerebral cortex, hippocampus and cerebellum. Myelination and synapse formation are intact. Abnormal myelinated fibers are present in the molecular layer of the cerebral cortex and in the central gray matter of the spinal cord. These morphological abnormalities resemble those reported in the reeler mutant mouse. SRK rats are another good animal model of human congenital malformations with neuronal migration disorders.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00686973
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  • 8
    Electronic Resource
    Electronic Resource
    Variability in the activity of respiratory chain enzymes in mitochondrial myopathies (1988)
    Springer
    Acta neuropathologica 76 (1988), S. 135-141 
    Details
    ISSN: 1432-0533
    Keywords: Mitochondrial myopathy ; Respiratory chain enzyme ; Cytochromec oxidase ; NADH-coenzyme Q reductase ; Ragged-red fiber
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Four patients with mitochondrial abnormality had multiple muscle biopsies at several year intervals during which respiratory chain enzyme activities were shown to be quite variable. In three patients, progression of the disease paralleled the decrease in respiratory chain enzyme activity. In one patient, the clinical and pathological findings improved with age as is seen in the benign infantile form of cytochromec oxidase (CCO) deficiency. The variability in these mitochondrial disorders may result from the varied proportions of normal and abnormal mitochondria in the muscle cells in which the mitochondria are said to be randomly replicated from numerous mitochondrial DNA copies.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00688097
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  • 9
    Electronic Resource
    Electronic Resource
    Myopathy in Marinesco-Sjögren syndrome: an ultrastructural study (1990)
    Springer
    Acta neuropathologica 80 (1990), S. 123-128 
    Details
    ISSN: 1432-0533
    Keywords: Marinesco-Sjögren syndrome ; Muscle biopsy ; Electron microscopy ; Autophagocytosis ; Double-membrane structure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Seven muscle biopsies from patients with the clinical characteristics of Marinesco-Sjögren syndrome (MSS) revealed myopathic changes of two types; muscle fiber necrosis followed by regeneration and focal myofibrillar degeneration inducing autophagocytosis with rimmed vacuole formation. In two young patients, massive muscle fiber necrosis with phagocytic invasion was the predominant feature and autophagic phenomenon was minimal, resembling the findings in progressive muscular dystrophy. Myofibrillar degeneration with autophagic phenomenon was prominent in five adult patients. The coexistence of these two degenerative processes and the secondarily induced reactive changes of muscle fiber hypertrophy, interstitial fibrosis, occasional ragged-red fibers and type 1 fiber predominance, are responsible for the wide spectrum of muscle pathology in MSS. The dense double-membrane structure surrounding myonuclei, previously reported as being specific to MSS, was present in only one biopsy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00308914
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  • 10
    Electronic Resource
    Electronic Resource
    Cytochrome c oxidase activity is deficient in blood vessels of patients with myoclonus epilepsy with ragged-red fibers (1993)
    Springer
    Acta neuropathologica 85 (1993), S. 280-284 
    Details
    ISSN: 1432-0533
    Keywords: Mitochondrial encephalomyopathy ; Myoclonus epilepsy with ragged-red fibers (MERRF) ; Strongly succinate dehydrogenase-reactive blood vessels (SSV) ; Cytochrome c oxidase (CCO) ; Vascular involvement
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary More than half of the intramuscular blood vessels in muscle biopsies from five patients with myoclonus epilepsy with ragged-fibers (MERRF) who had a point mutation in mitochondrial DNA at the tRNALys region were darkly stained with succinate dehydrogenase (SDH) stain, showing the morphologic characteristics of strongly SDH-reactive blood vessels (SSV), but they had no cytochrome c oxidase (CCO) activity. By electron cytochemistry, the mitochondria in the smooth muscle cells of SSV had no CCO activity. On the other hand, SSV in muscle biopsies from patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) had normal CCO activity as shown by light and electron microscopy. The defect in CCO activity in the arteriolar smooth muscle cells and in muscle fibers suggests that CCO deficiency is related to the pathophysiology of MERRF.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00227723
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