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  • 1
    ISSN: 1432-0533
    Keywords: Chromatolytic neurons ; Werdnig-Hoffmann disease ; Immunocytochemistry ; Phosphorylated neurofilament ; Sudden infant death syndrome (SIDS)
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Two cases of Werdnig-Hoffman disease are described. Chromatolytic neurons were found within the ventral horns of the spinal cord as well as in dorsal root ganglia, Clarke's column, cranial nerve nuclei and thalamus. Immunohistochemical staining demonstrated ohosphorylated neurofilament antigen within the cytoplasm of the chromatolytic neurons. This finding suggests that a defect in slow axonal transport or in the regulation of neurofilament phosphorylation may play a role in the pathogenesis of this disorder.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Immunocytochemistry ; Synapse ; Synapsin I ; Tuber ; Tuberous sclerosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary To evaluate the effects of tuberous sclerosis (TS) on cortical synaptic organization, we analyzed synaptic densities within cortical tubers from the brains of two TS patients using a polyclonal antibody directed against synapsin I, a synaptic terminal-specific protein. The synaptic densities of the tubers and adjacent histologically normal cortex were obtained by determining optical densities using an IBAS (Zeiss) image analysis system. The tubers showed abnormally low levels of synapsin I compared with the normal cortex. The data suggest that cortical tubers do not contain a normal complement of synapses. This may reflect focal underdevelopment of normal cortical-cortical connections. Altered afferent corticla projections may also contribute to synaptic loss in cortical tubers.
    Type of Medium: Electronic Resource
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