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  • Electronic Resource  (3)
  • Cholesterol metabolism  (3)
  • 1
    Electronic Resource
    Electronic Resource
    Peripheral nerve pathology in Niemann-Pick type C mouse (1995)
    Springer
    Acta neuropathologica 90 (1995), S. 158-163 
    Details
    ISSN: 1432-0533
    Keywords: Key words Schwann cell ; Paranodal region ; Cholesterol metabolism ; Myelin remodeling ; Myelin maintenance
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The sciatic nerve of the mouse mutant with Niemann-Pick type C disease (NPC mouse) was investigated using light and electron microscopy, and teased-fiber preparations. As early as postnatal day 20, when clinical symptoms were not yet apparent, focal paranodal swellings with an accumulation of small myelin figures in the Schwann cell cytoplasm were noted. These paranodal changes were more pronounced in the distal segment and became progressively conspicuous with increasing age. The morphometric analysis revealed a hypomyelination of large myelinated fibers in the NPC nerves at 70 days, whereas an essentially similar histogram pattern was noted in both control and NPC nerves at 20 days, suggesting progressively defective utilization of cholesterol in the NPC nerves with age. Intraxonal accumulation of dense bodies was noted in older mice, but no segmental demyelination or Wallerian type of axonal degeneration was observed at any age. The changes noted in the paranodal regions in the NPC mouse closely resemble those found in rats treated with an inhibitor of cholesterol biosynthesis, as well as those seen in remodeling fibers during an early stage of peripheral nerve development. Thus, the morphological changes seen in the sciatic nerve of the NPC mouse may be an expression of perturbation in myelin maintenance as a result of defective cholesterol metabolism.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294315
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
  • 2
    Electronic Resource
    Electronic Resource
    Peripheral nerve pathology in Niemann-Pick type C mouse (1995)
    Springer
    Acta neuropathologica 90 (1995), S. 158-163 
    Details
    ISSN: 1432-0533
    Keywords: Schwann cell ; Paranodal region ; Cholesterol metabolism ; Myelin remodeling ; Myelin maintenance
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The sciatic nerve of the mouse mutant with Niemann-Pick type C disease (NPC mouse) was investigated using light and electron microscopy, and teased-fiber preparations. As early as postnatal day 20, when clinical symptoms were not yet apparent, focal paranodal swellings with an accumulation of small myelin figures in the Schwann cell cytoplasm were noted. These paranodal changes were more pronounced in the distal segment and became progressively conspicuous with increasing age. The morphometric analysis revealed a hypomyelination of large myelinated fibers in the NPC nerves at 70 days, whereas an essentially similar histogram pattern was noted in both control and NPC nerves at 20 days, suggesting progressively defective utilization of cholesterol in the NPC nerves with age. Intraxonal accumulation of dense bodies was noted in older mice, but no segmental demyelination or Wallerian type of axonal degeneration was observed at any age. The changes noted in the paranodal regions in the NPC mouse closely resemble those found in rats treated with an inhibitor of cholesterol biosynthesis, as well as those seen in remodeling fibers during an early stage of peripheral nerve development. Thus, the morphological changes seen in the sciatic nerve of the NPC mouse may be an expression of perturbation in myelin maintenance as a result of defective cholesterol metabolism.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294315
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 3
    Electronic Resource
    Electronic Resource
    Neurofibrillary tangles in Niemann-Pick disease type C (1995)
    Springer
    Acta neuropathologica 89 (1995), S. 227-238 
    Details
    ISSN: 1432-0533
    Keywords: Neuronal storage disease ; Cholesterol metabolism ; Tau ; Paired helical filaments ; Lysosomal disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Niemann-Pick disease type C (NPC) is an autosomal recessive disease, belonging to a clinically heterogeneous group of lipid storage diseases, distinguished by a unique error in cellular trafficking of exogenous cholesterol, associated with lysosomal accumulation of unesterified cholesterol. Unlike Niemann-Pick disease types A and B, there is no primary genetic defect in sphingomyelinase in NPC. During the routine neuropathological study of NPC patients, we found neurofibrillary tangles (NFT) in a series of cases with a slowly progressive chronic course. These were not associated with β-amyloid deposits. The NFT were most frequent in the orbital gyrus, cingulate gyrus and entorhinal region of the cerebral cortex, but were also frequently found in the basal ganglia, thalamus and hypothalamus. In one of the most severely affected case, the NFT were even found in the neurons in the inferior olivary nucleus and in the spinal cord. The NFT were immunostained with Alz 50, and cosisted of paired helical filaments. The distribution of the neurons bearing the NFT was generally similar to that of the swollen storage neurons, and storage neurons often contained NFT in their perikarya and/or in the meganeurites. However, neurons with NFT could be noted without swollen perikarya. The coexistence of neuronal storage and NFT in NPC without amyloid deposits suggests that perturbed cholesterol metabolism and/or lysosomal membrane trafficking may play a role in the formation of NFT, and that amyloid deposits are not necessarily the prerequisite for NFT formation. The results of our study also suggest that NFT formation may be a rather nonspecific cellular reaction of neurons to certain slowly progressive metabolic perturbations of an as yet undefined nature.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00309338
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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