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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 80 (1990), S. 541-546 
    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
    Library Location Call Number Volume/Issue/Year Availability
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  • 2
    Electronic Resource
    Electronic Resource
    Oxford, U.K. and Cambridge, USA : Blackwell Science Ltd
    Scandinavian journal of immunology 43 (1996), S. 0 
    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
    Library Location Call Number Volume/Issue/Year Availability
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