| Whole body cholesterol metabolism is impaired in Huntington's disease. | |
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MedLine Citation:
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PMID: 21406216 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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We previously reported impaired cholesterol biosynthesis in rodent Huntington Disease (HD) models and HD patients' fibroblasts and post mortem brains. We also found that plasma levels of 24S-hydroxycholesterol (24OHC), the brain specific elimination product of cholesterol considered a marker of brain cholesterol turnover, were significantly reduced in HD patients at any disease stage. In the present study we analysed by mass spectrometry the fasting plasma levels of cholesterol, its biosynthetic precursors lanosterol and lathosterol, of the whole-body elimination products 27-hydroxycholesterol and of brain 24OHC in a cohort of premanifest and HD patients at different disease stages. We found that the cholesterol precursors lanosterol and lathosterol (both index of whole body cholesterol synthesis), the levels of the bile acid precursor 27-hydroxycholesterol, and of the brain specific 24OHC, were all significantly reduced in manifest HD patients, suggesting that whole-body and brain cholesterol homeostasis are both impaired in HD. |
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Authors:
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V Leoni; C Mariotti; L Nanetti; E Salvatore; F Squitieri; A R Bentivoglio; M Bandettini di Poggio; M Bandettini Del Poggio; S Piacentini; D Monza; M Valenza; E Cattaneo; S Di Donato |
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Publication Detail:
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Type: Journal Article Date: 2011-03-22 |
Journal Detail:
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Title: Neuroscience letters Volume: 494 ISSN: 1872-7972 ISO Abbreviation: Neurosci. Lett. Publication Date: 2011 May |
Date Detail:
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Created Date: 2011-04-15 Completed Date: 2011-08-17 Revised Date: 2011-09-07 |
Medline Journal Info:
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Nlm Unique ID: 7600130 Medline TA: Neurosci Lett Country: Ireland |
Other Details:
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Languages: eng Pagination: 245-9 Citation Subset: IM |
Copyright Information:
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Copyright © 2011 Elsevier Ireland Ltd. All rights reserved. |
Affiliation:
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Laboratory of Clinical Pathology and Medical Genetics, Fondazione IRCCS Istituto Neurologico 'Carlo Besta', Via Celoria 11, 20133 Milano, MI, Italy. valerioleoni@hotmail.com |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Brain / metabolism* Cholesterol / metabolism* Female Humans Huntington Disease / metabolism* Male Middle Aged |
| Chemical | |
Reg. No./Substance:
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57-88-5/Cholesterol |
| Comments/Corrections | |
Erratum In:
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Neurosci Lett. 2011 Jul 8;498(2):171 Note: Bandettini Del Poggio, M [corrected to Bandettini di Poggio, M] |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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