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  • 1
    ISSN: 1432-0533
    Keywords: Heparan sulfate proteoglycan ; Neurofilament ; Spheroid ; Conglomerate inclusion ; Motor neuron disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The spinal cords from eight autopsy cases of sporadic motor neuron disease (MND) and two control cases were immunohistochemically examined using anti-bodies directed to neurofilament proteins (anti-Nf) and to heparan sulfate (HepSS-1). Variable numbers of spheroids were observed in the anterior horns in the MND cases. In one case of MND, one third to half of the remaining anterior horn cells contained conglomerate inclusions in their perikarya. These pathological structures were not encountered in the control cases. The immunohistochemical study revealed that both anti-Nf and HepSS-1 intensely labelled all spheroids and conglomerate inclusions in the MND cases. The colocalization of heparan sulfate with neurofilamentous accumulation suggests that heparan sulfate is required for the aggregation of neurofilaments, resulting in the formation of spheroids and conglomerate inclusions in MND.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Neurochemical research 13 (1988), S. 621-624 
    ISSN: 1573-6903
    Keywords: Neurofilament proteins ; axonal type ; perikaryal type ; phosphorylation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In previous studies, neuronal cell bodies, excised by hand from bovine spinal ganglia, were analyzed and heterogeneous intermediate and high molecular weight neurofilament proteins that differed in electrophoretic mobility from their axonal counterparts were demonstrated (1, 2). In the present experiment, intermediate and high molecular weight neurofilament proteins of the axonal type were treated with alkaline phosphatase, and neurofilament proteins enriched in perikaryal type proteins were labeled with32P. Results showed that neurofilament proteins were phosphorylated after their translation, in the perikarya and the proximal portion of the axon, and suggested that phosphorylation was responsible for the differences between axonal and perikaryal neurofilament proteins.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Peripheral myelin protein22 (PMP22), a membrane glycoprotein, plays a significant role in the formation and/or maintenance of compact myelin in the peripheral nervous system. We studied two pedigrees with Dejerine-Sottas disease and identified two novel mutations in the PMP22 gene: one a 2-bp deletional mutation at nucleotide positions426 and 427 of exon4 (this is predicted to alter the reading frame at leucine80 and thus to lead to frame-shifted translation), and the other a guanine to thymine substitution at nucleotide position636 leading to a cysteine substitution for glycine150. Both mutations were located in the putative transmembrane domains reported in many cases of Charcot-Marie-Tooth neuropathy, Dejerine-Sottas disease, and hereditary neuropathy with liability to pressure palsies. The results suggest an important role for the putative transmembrane domains of PMP22 in its function.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1435-5922
    Keywords: fulminant hepatitis ; hepatitis C virus ; multiple sclerosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 55-year-old woman was treated at our hospital for multiple sclerosis. Therapy consisted of glucocorticosteroids and cyclosporin. In the 7th week after these drugs were discontinued the patient developed acute liver failure due to fulminant hepatitis (FH) and died. Post-mortem examination showed massive liver necrosis. Serologic examination was negative for hepatitis B virus-related markers. Anti-hepatitis C virus (anti-HCV) antibody and serum HCV RNA were negative on admission, but HCV RNA appeared concurrently with the onset of FH. Although HCV infection rarely causes FH, it was considered to be the cause of FH in this patient, since there were no other causes of acute liver injury. We suspect that underlying immunologic abnormalities in conjunction with HCV infection may have precipitated the FH.
    Type of Medium: Electronic Resource
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