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  • 1
    Electronic Resource
    Electronic Resource
    Inclusion Body Myositis: Clonal Expansions of Muscle-Infiltrating T Cells Persist Over Time (2003)
    Oxford, UK : Blackwell Science Ltd
    Scandinavian journal of immunology 58 (2003), S. 0 
    Details
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Inclusion body myositis (IBM) is a chronic inflammatory myopathy. The muscle histology is characterized by infiltration of T cells, which invade and apparently destroy muscle fibres. This study was performed to investigate whether predominant clones of muscle-infiltrating T cells are identical in different muscles and whether they persist over time in IBM. By reverse transcriptase-polymerase chain reaction, 25 T-cell receptor (TCR) variable β (Vβ) chain families and the complementarity-determining region 3 (CDR3) of the TCR were analysed in two different muscle biopsies of four patients with IBM. In two of the patients, the muscle biopsies were obtained from different muscles at one time point, whereas in two patients, the second biopsy was obtained 9 years after the first biopsy. T cells expressing predominant Vβ families were analysed for clonality by fragment length analysis of the CDR3. Predominant Vβ families were analysed by DNA sequencing to identify identical clones. Immunohistochemical staining of Vβ families was performed to study the distribution of T cells expressing identified predominant Vβ families. The muscle-infiltrating lymphocytes showed restricted expression of TCR Vβ families. DNA sequencing proved that clonally expanded T cells were identical in different muscles and persisted 9 years after the first biopsy. Immunohistochemical analysis with Vβ family-specific antibodies demonstrated the endomysial localization of these T cells in inflammatory cell infiltrates. Our results show that in IBM there is clonal restriction of TCR expression in muscle-infiltrating lymphocytes. Identical T-cell clones predominate in different muscles, and these clones persist for many years. These results indicate an important, continuous, antigen-driven inflammatory reaction in IBM.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-3083.2003.01251.x
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  • 2
    Electronic Resource
    Electronic Resource
    Limited T-Cell Receptor V Gene Usage in Inclusion Body Myositis (1996)
    Oxford, U.K. and Cambridge, USA : Blackwell Science Ltd
    Scandinavian journal of immunology 43 (1996), S. 0 
    Details
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Inclusion body myositis (IBM) is a chronic, progressive inflammatory myopathy. The inflammatory infiltrates are dominated by T cells, which frequently invade muscle fibres. The present study was performed to characterize the usage of the variable (V)segment of the T-cell receptor of muscle infiltrating cells in IBM. Using the reverse transcriptase polymerase chain reaction (RT-PCR) technique the authors analysed the expression of 22 Vα and 24 Vβ families in muscle tissue from six patientswith IBM displaying intense inflammatory cell infiltration. The following Vα/Vβ families appeared in at least 50% of the patients: Vα1, 5, 7, 15, 16, 17, 20, 21, 22 and Vβ3, 5.2, 8, 12, 14, 22. In all patients Vα7, 16 and Vβ8 wereexpressed in muscle tissue. Furthermore, in two of the patients peripheral blood lymphocytes (PBL) were investigated in parallel. There was a restricted usage of Vα and Vβ families in muscle in comparison to PBL, indicating a selective homing orlocal proliferation of T lymphocytes in the inflammatory lesions in IBM.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-3083.1996.d01-10.x
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  • 3
    Electronic Resource
    Electronic Resource
    Local T-Cell Proliferation and Differentiation in Inflammatory Myopathies (1995)
    Oxford, UK : Blackwell Publishing Ltd
    Scandinavian journal of immunology 41 (1995), S. 0 
    Details
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Our objective was to investigate the patterns of proliferation and differentiation of infiltrating cells in inflammatory myopathies. Immunohistochemical staining was performed on muscle biopsy specimens from 18 patients with inclusion body myositis, polymyositis and dermatomyositis using monoclonal and polyclonal antibodies.An abundance of cells were TNF-α+ (4–8%), ICAM-1+ (7–65%). IFN-γ+ (3–6%), and Ki-67+ (4–8%). It was shown that 70% of the Ki-67+ cells were Ki-67+CD3+ cells. Very few mononuclear cells were IL-2R+. MHC-I expression was found on nearly all muscle fibres in all cases, while MHC-II expression was found on occasional muscle fibres in 1/3 of cases. Analysis of repeated biopsies from four IBM patients after prednisolone treatment showed no change in the proportions of TNF-α, ICAM-1, IFN-γ or Ki-67 positive cells. In inflammatory myopathies there is an intense proliferation and differentiation of inflammatory cells in situ, indicating a local stimulation of the inflammatory process.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-3083.1995.tb03587.x
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  • 4
    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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  • 5
    Electronic Resource
    Electronic Resource
    Automatic sequencing of mitochondrial tRNA genes in patients with mitochondrial encephalomyopathy
    Amsterdam : Elsevier
    Biochimica et Biophysica Acta (BBA)/Molecular Basis of Disease 1226 (1994), S. 49-55 
    Details
    ISSN: 0925-4439
    Keywords: Mitochondria ; Myopathy ; encephalomyopathy ; mtDNA ; tRNA
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Medicine , Physics
    Type of Medium: Electronic Resource
    URL: http://linkinghub.elsevier.com/retrieve/pii/0925-4439(94)90058-2
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  • 6
    Electronic Resource
    Electronic Resource
    Low Levels of Mitochondrial Transcription Factor A in Mitochondrial DNA Depletion
    Amsterdam : Elsevier
    Biochemical and Biophysical Research Communications 200 (1994), S. 1374-1381 
    Details
    ISSN: 0006-291X
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Physics
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1006/bbrc.1994.1603
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  • 7
    Electronic Resource
    Electronic Resource
    Neuropathology in Kearns-Sayre syndrome (1990)
    Springer
    Acta neuropathologica 80 (1990), S. 541-546 
    Details
    ISSN: 1432-0533
    Keywords: Mitochondria ; Central nervous system ; Skeletal muscle ; Pathology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The neuropathological changes found at autopsy in a case of Kearns-Sayre syndrome are described. We have previously analyzed the respiratory chain function in isolated muscle mitochondria and also described a large deletion of muscle mitochondrial DNA (mtDNA) in this case. The neuropathological examination revealed prominent neuronal degeneration and gliosis of the basal ganglia and there were bilateral areas of softening and total loss of nerve cells in the lenticular nuclei. The pallidum and caudate nucleus disclosed accumulation of iron-containing pigment. The white matter in the cerebrum, brain stem and cerebellum showed widespread and focally accentuated spongy change due to splitting of myclin lamellae. It is suggested that deficiency of respiratory chain enzymes due to the mtDNA deletion is of pathogenetic importance in the development of the described changes.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294616
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  • 8
    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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  • 9
    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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  • 10
    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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