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  • 1
    Electronic Resource
    Electronic Resource
    Immunohistochemical demonstration of different muscle fibre types in paraffin sections (1989)
    Oxford, UK : Blackwell Publishing Ltd
    Histopathology 15 (1989), S. 0 
    Details
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2559.1989.tb01594.x
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  • 2
    Electronic Resource
    Electronic Resource
    Muscle regeneration (1989)
    Springer
    Acta neuropathologica 78 (1989), S. 264-269 
    Details
    ISSN: 1432-0533
    Keywords: Skeletal muscle ; Regeneration ; Growth factors ; Growth hormone ; Rat
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Against the background of the importance of growth hormone (GH) for normal muscle growth, a study was performed to investigate whether lack of GH after hypophysectomy affects the cell proliferation and the local production of insulin-like growth factor-I (IGF-I) in the early stages of muscle regeneration in adult rats. The level of IGF-I in the serum of hypophysectomised rats was reduced to about 30% of that of controls. The incorporation of [methyl-3H]thymidine into the regenerating muscle showed a peak 6 days after the operation and then gradually declined to the end of the period of study 30 days after initiation of regeneration by ischemic necrosis. The DNA content rose to a maximum level after 6–8 days, and remained high after 30 days. There was no major difference in the incorporation of [3H]thymidine in regenerating muscle of hypophysectomised and control rats, but the DNA concentration in the regenerating muscles of hypophysectomised rats was significantly reduced after 30 days. There was a corresponding reduction in the number of nuclei per muscle fibre, indicating that hypophysectomy has a small effect on the cell proliferation during the early stages of muscle regeneration. Immunohistochemical demonstration of IGF-I in the regenerating muscle revealed the transient presence of immunoreactive material in satellite cells and myotubes after 6 to 8 days of regeneration but no immunoreactivity after 30 days. No obvious difference was observed between hypophysectomised and control rats, indicating that the endogenous production of IGF-I in regenerating skeletal muscle can occur independently of GH.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687756
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  • 3
    Electronic Resource
    Electronic Resource
    Mitochondrial encephalomyopathy (1987)
    Springer
    Acta neuropathologica 74 (1987), S. 287-293 
    Details
    ISSN: 1432-0533
    Keywords: Mitochondrial encephalomyopathy ; Lactic acidosis ; Stroke-like episodes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We report the clinical and autopsy findings in a young man of 18 with a chronic progressive disorder comprised of lactic acidosis, mental deterioration, and epileptic seizures which were sometimes accompanied by stroke-like episodes with transient hemiparesis and cortical blindness. He died of congestive heart failure. The autopsy showed lesions of the gray matter of the brain. Both the putamen and parieto-occipital cortex showed loss of neurons and proliferation of macrophages, astrocytes and vessels. There was marked loss of neurons in the inferior olives, and slight reduction of the number of Purkinje cells. Skeletal muscle studies revealed ragged-red fibres and structurally abnormal mitochondria. The heart was enlarged: accumulations of mitochondria occurred in the muscle fibers. The liver exhibited marked fatty degeneration. Biochemical analyses showed normal activities of pyruvate dehydrogenase in thrombocytes, pyruvate carboxylase in lymphocytes, biotinidase in serum as well as succinate dehydrogenase and cytochrome c oxidase. The features of this disorder differ in many respects from cases of mitochondrial encephalomyopathy previously reported and cannot be assigned to any specific disease entity.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00688194
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  • 4
    Electronic Resource
    Electronic Resource
    Neuropathology of Salla disease (1988)
    Springer
    Acta neuropathologica 75 (1988), S. 481-490 
    Details
    ISSN: 1432-0533
    Keywords: Sialuria ; Salla disease ; CNS pathology ; Neurofibrillary tangles
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A neuropathological study was performed on two patients with Salla disease, one male and one female, from different families. They both died at the age of 41 years. Both patients showed increased excretion of free sialic acid in the urine, psychomotor retardation starting in the 1st year of life, ataxia and spasticity. Several family members of both families were affected with the same disease indicating the hereditary character of the disorder. The neuropathological investigation revealed strikingly similar changes in the two cases. Macroscopically the cerebral white matter was severely reduced. Histologically marked loss of axons and myelin sheaths was accompanied by pronounced astrocytic proliferation. The remaining axons frequently showed ovoid swellings surrounded by a myelin sheath. The reduction of the number of myelin sheaths seemed proportional to the numerical reduction of axons. Many cortical nerve cells displayed in relation to age an abnormal amount of lipofuscin. Neurofibrillary tangles were observed in nerve cells of the neo-cortex, nucleus basalis of Meynert and locus ceruleus. Cerebellum showed moderate loss of Purkinje cells. In the spinal cord axonal degeneration was observed in both ascending and descending tracts.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687135
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  • 5
    Electronic Resource
    Electronic Resource
    Cytochromec oxidase deficiency in infancy (1989)
    Springer
    Acta neuropathologica 77 (1989), S. 267-275 
    Details
    ISSN: 1432-0533
    Keywords: Mitochondrial myopathy ; Respiratory chain enzyme ; Enzyme-histochemistry ; Cytochromec oxidase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Five children with early onset of muscle weakness, lactic acidosis and deficient cytochromec oxidase staining in the muscle biopsy were studied. By oximetric assay of the respiratory chain of isolated mitochondria, cytochromec oxidase deficiency was confirmed in four of the cases, while one case showed only a slight decrease of cytochromec oxidase activity but considerably reduced activity when assayed spectrophotometrically. The muscle biopsies exhibited mitochondrial structural abnormalities and lipid storage in the four cases with oximetrically confirmed cytochromec oxidase deficiency, while the biopsy of the case with markedly reduced activity of cytochromec oxidase only in the enzyme-histochemical and spectrophotometrical assays had normal morphology. The light microscopical staining of cytochromec oxidase in the four cases with oximetrically confirmed deficiency showed deficient staining of the enzyme in all extrafusal fibres in three cases but one of the cases had normal enzyme-histochemical activity of cytochromec oxidase in about 25% of the fibres. In two cases muscle spindles were included in the biopsy. The intrafusal fibres showed normal enzyme-histochemical activity of cytochromec oxidase. Ultrastructural examination of the enzyme distribution in two of the cases revealed great heterogeneity of the mitochondria. The structurally abnormal mitochondria were usually deficient of enzyme activity. The mitochondria of endothelial cells appeared to have normal activity. Immunohistochemical staining with polyclonal antibodies to cytochromec oxidase revealed presence of immunoreactive material corresponding to the localisation of mitochondria in all cases. The results show that enzyme-histochemical staining of cytochromec oxidase is a useful technique to reveal deficiency of the enzyme and to study the distribution of the deficiency within the tissue both at the light microscopical and ultrastructural levels. However, the results of one of the cases show that deficiency revealed by the enzyme-histochemical technique is not completely reliable. Oximetric studies on isolated mitochondria are necessary to confirm the suspected deficiency and to reveal combined defects of the respiratory chain.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687578
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  • 6
    Electronic Resource
    Electronic Resource
    Autofluorescence emission spectra of neuronal lipopigment in mucopolysaccharidosis (Sanfilippo's syndrome) (1989)
    Springer
    Acta neuropathologica 77 (1989), S. 426-429 
    Details
    ISSN: 1432-0533
    Keywords: Mucopolysaccharidosis ; Sanfilippo's syndrome ; Ceroid ; Lipofuscin ; Autofluorescence
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Characteristics of intraneuronal lipopigment in two siblings with Sanfilippo's syndrome are reported. A lipopigment emission spectrum probably reflects its composition and the (uncorrected) autofluorescence emission spectra results are compared with spectra from non-diseased tissue and from previously reported childhood-onset neuronal ceroidlipofuscinoses (ceroidoses), adult-onset ceroidosis (Kufs' disease) and animal ceroidoses. Values derived from the emission spectra from Sanfilippo's syndrome could be distinguished from those obtained from equivalent regions of non-diseased brains and were within the range of abnormal values previously reported from accumulations of pigment in various types of neuronal ceroidosis. Some abnormal lipopigment in Sanfilippo tissue was indistinguishable from some lipopigment in childhood-onset ceroidosis and in Kufs' disease. These results indicate that the intraneuronal lipopigment which accumulates to an abnormal extent in Sanfilippo's syndrome should not be termed “lipofuscin”, which is a normal cerebral constituent, but “ceroid” to denote lipopigment with abnormal characteristics.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687378
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  • 7
    Electronic Resource
    Electronic Resource
    Cholera toxin B-subunit incorporation into synaptic vesicles of the neuromuscular junction of the rat (1986)
    Springer
    Cellular and molecular life sciences 42 (1986), S. 415-417 
    Details
    ISSN: 1420-9071
    Keywords: Synaptic vesicles ; endocytosis ; cholera toxin ; ganglioside ; neuromuscular junction ; rat
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary The B-subunit of cholera toxin, a nontoxic macromolecule which binds specifically to GM1 ganglioside, was conjugated to colloidal gold and injected into skeletal muscle of the rat. It was taken up rapidly in vesicles in the terminal axons at the neuromuscular junctions. Injection of albumin-colloidal gold conjugates resulted in an insignificant uptake. The results indicate that uptake of extracellular macromolecules into the terminal axon of the neuromuscular junction may be greatly enhanced by binding to gangliosides at the presynaptic membrane, and that it may occur without association with vesicular recycling related to transmitter release.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02118636
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