Cholesterol storage and tau pathology in Niemann–Pick type C disease in the brain
Identifieur interne : 002122 ( Main/Exploration ); précédent : 002121; suivant : 002123Cholesterol storage and tau pathology in Niemann–Pick type C disease in the brain
Auteurs : Roland Distl [Allemagne] ; Stephanie Treiber-Held [Allemagne] ; Frank Albert [Allemagne] ; Volker Meske [Allemagne] ; Klaus Harzer [Allemagne] ; Thomas G. Ohm [Allemagne]Source :
- The Journal of Pathology [ 0022-3417 ] ; 2003-05.
Abstract
Niemann–Pick type C disease is an inherited neurovisceral storage disorder with intracellular accumulation of cholesterol. In affected brains, many ballooned neurons are seen. Considerable nerve cell loss of unknown pathogenesis leads to neurological deterioration and dementia. Chemical examination of brains has failed to demonstrate increased levels of cholesterol. Using filipin fluorometry of neuronal cells in tissue slices, we found massive accumulation of cholesterol in neurons in four out of five human Niemann–Pick type C cases including adult patients. Neurofibrillary tangles composed of aggregates of the otherwise highly soluble protein tau were present in three Niemann–Pick type C cases and were also immunologically identical to those associated with Alzheimer's disease. However, only a thin slab of spinal cord or a tiny piece of isocortex was available for examination in the two cases without tangles. In a further semi‐quantitative analysis of 576 neurons, we determined higher cholesterol content in tangle‐bearing neurons than in adjacent tangle‐free neurons. The association of cholesterol accumulation with neurofibrillary degeneration in Niemann–Pick type C disease and Alzheimer's disease awakens interest in the role of impaired cholesterol metabolism in the development of neurofibrillary tangles in both diseases. Copyright © 2003 John Wiley & Sons, Ltd.
Url:
DOI: 10.1002/path.1320
Affiliations:
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In affected brains, many ballooned neurons are seen. Considerable nerve cell loss of unknown pathogenesis leads to neurological deterioration and dementia. Chemical examination of brains has failed to demonstrate increased levels of cholesterol. Using filipin fluorometry of neuronal cells in tissue slices, we found massive accumulation of cholesterol in neurons in four out of five human Niemann–Pick type C cases including adult patients. Neurofibrillary tangles composed of aggregates of the otherwise highly soluble protein tau were present in three Niemann–Pick type C cases and were also immunologically identical to those associated with Alzheimer's disease. However, only a thin slab of spinal cord or a tiny piece of isocortex was available for examination in the two cases without tangles. In a further semi‐quantitative analysis of 576 neurons, we determined higher cholesterol content in tangle‐bearing neurons than in adjacent tangle‐free neurons. The association of cholesterol accumulation with neurofibrillary degeneration in Niemann–Pick type C disease and Alzheimer's disease awakens interest in the role of impaired cholesterol metabolism in the development of neurofibrillary tangles in both diseases. Copyright © 2003 John Wiley & Sons, Ltd.</div></front></TEI><affiliations><list><country><li>Allemagne</li></country><region><li>Bade-Wurtemberg</li><li>Berlin</li><li>District de Tübingen</li></region><settlement><li>Berlin</li><li>Tübingen</li></settlement></list><tree><country name="Allemagne"><region name="Berlin"><name sortKey="Distl, Roland" sort="Distl, Roland" uniqKey="Distl R" first="Roland" last="Distl">Roland Distl</name></region><name sortKey="Albert, Frank" sort="Albert, Frank" uniqKey="Albert F" first="Frank" last="Albert">Frank Albert</name><name sortKey="Harzer, Klaus" sort="Harzer, Klaus" uniqKey="Harzer K" first="Klaus" last="Harzer">Klaus Harzer</name><name sortKey="Meske, Volker" sort="Meske, Volker" uniqKey="Meske V" first="Volker" last="Meske">Volker Meske</name><name sortKey="Ohm, Thomas G" sort="Ohm, Thomas G" uniqKey="Ohm T" first="Thomas G" last="Ohm">Thomas G. 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