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Analysis by Liquid Chromatography - Mass Spectrometry of Sterols and Oxysterols in Brain of the Newborn Dhcr7Δ3-5/T93M Mouse: A Model of Smith-Lemli-Opitz Syndrome

Anna Meljon, Gordon L Watson, Yuqin Wang Orcid Logo, Cedric H.L Shackleton, William Griffiths Orcid Logo

Biochemical Pharmacology

Swansea University Authors: Yuqin Wang Orcid Logo, William Griffiths Orcid Logo

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DOI (Published version): 10.1016/j.bcp.2013.03.003

Abstract

In this study the sterol and oxysterol profile of newborn brain from the Dhcr7Δ3-5/T93M mouse model of Smith-Lemli-Opitz syndrome (SLOS) has been investigated. This is a viable mouse model which is compound heterozygous containing one null allele and one T93M mutation on Dhcr7. We find the SLOS mous...

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Published in: Biochemical Pharmacology
ISSN: 0006-2952
Published: 2013
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URI: https://cronfa.swan.ac.uk/Record/cronfa14485
first_indexed 2013-07-23T12:12:18Z
last_indexed 2018-02-09T04:45:53Z
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spelling 2013-08-01T08:01:09.5847727 v2 14485 2013-03-26 Analysis by Liquid Chromatography - Mass Spectrometry of Sterols and Oxysterols in Brain of the Newborn Dhcr7Δ3-5/T93M Mouse: A Model of Smith-Lemli-Opitz Syndrome c92729b58622f9fdf6a0e7d8f4ce5081 0000-0002-3063-3066 Yuqin Wang Yuqin Wang true false 3316b1d1b524be1831790933eed1c26e 0000-0002-4129-6616 William Griffiths William Griffiths true false 2013-03-26 MEDS In this study the sterol and oxysterol profile of newborn brain from the Dhcr7Δ3-5/T93M mouse model of Smith-Lemli-Opitz syndrome (SLOS) has been investigated. This is a viable mouse model which is compound heterozygous containing one null allele and one T93M mutation on Dhcr7. We find the SLOS mouse has reduced levels of cholesterol and desmosterol and increased levels of 7- and 8-dehydrocholesterol and of 7- and 8-dehydrodesmosterol in brain compared to the wild type. The profile of enzymatically formed oxysterols in the SLOS mouse resembles that in the wild type but the level of 24S-hydroxycholesterol, the dominating cholesterol metabolite, is reduced in a similar proportion to that of cholesterol. A number of oxysterols abundant in the SLOS mouse are probably derived from 7-dehydrocholesterol, however, the mechanism of their formation is unclear. Journal Article Biochemical Pharmacology 0006-2952 31 12 2013 2013-12-31 10.1016/j.bcp.2013.03.003 COLLEGE NANME Medical School COLLEGE CODE MEDS Swansea University 2013-08-01T08:01:09.5847727 2013-03-26T13:52:03.4937879 Faculty of Medicine, Health and Life Sciences Swansea University Medical School - Medicine Anna Meljon 1 Gordon L Watson 2 Yuqin Wang 0000-0002-3063-3066 3 Cedric H.L Shackleton 4 William Griffiths 0000-0002-4129-6616 5
title Analysis by Liquid Chromatography - Mass Spectrometry of Sterols and Oxysterols in Brain of the Newborn Dhcr7Δ3-5/T93M Mouse: A Model of Smith-Lemli-Opitz Syndrome
spellingShingle Analysis by Liquid Chromatography - Mass Spectrometry of Sterols and Oxysterols in Brain of the Newborn Dhcr7Δ3-5/T93M Mouse: A Model of Smith-Lemli-Opitz Syndrome
Yuqin Wang
William Griffiths
title_short Analysis by Liquid Chromatography - Mass Spectrometry of Sterols and Oxysterols in Brain of the Newborn Dhcr7Δ3-5/T93M Mouse: A Model of Smith-Lemli-Opitz Syndrome
title_full Analysis by Liquid Chromatography - Mass Spectrometry of Sterols and Oxysterols in Brain of the Newborn Dhcr7Δ3-5/T93M Mouse: A Model of Smith-Lemli-Opitz Syndrome
title_fullStr Analysis by Liquid Chromatography - Mass Spectrometry of Sterols and Oxysterols in Brain of the Newborn Dhcr7Δ3-5/T93M Mouse: A Model of Smith-Lemli-Opitz Syndrome
title_full_unstemmed Analysis by Liquid Chromatography - Mass Spectrometry of Sterols and Oxysterols in Brain of the Newborn Dhcr7Δ3-5/T93M Mouse: A Model of Smith-Lemli-Opitz Syndrome
title_sort Analysis by Liquid Chromatography - Mass Spectrometry of Sterols and Oxysterols in Brain of the Newborn Dhcr7Δ3-5/T93M Mouse: A Model of Smith-Lemli-Opitz Syndrome
author_id_str_mv c92729b58622f9fdf6a0e7d8f4ce5081
3316b1d1b524be1831790933eed1c26e
author_id_fullname_str_mv c92729b58622f9fdf6a0e7d8f4ce5081_***_Yuqin Wang
3316b1d1b524be1831790933eed1c26e_***_William Griffiths
author Yuqin Wang
William Griffiths
author2 Anna Meljon
Gordon L Watson
Yuqin Wang
Cedric H.L Shackleton
William Griffiths
format Journal article
container_title Biochemical Pharmacology
publishDate 2013
institution Swansea University
issn 0006-2952
doi_str_mv 10.1016/j.bcp.2013.03.003
college_str Faculty of Medicine, Health and Life Sciences
hierarchytype
hierarchy_top_id facultyofmedicinehealthandlifesciences
hierarchy_top_title Faculty of Medicine, Health and Life Sciences
hierarchy_parent_id facultyofmedicinehealthandlifesciences
hierarchy_parent_title Faculty of Medicine, Health and Life Sciences
department_str Swansea University Medical School - Medicine{{{_:::_}}}Faculty of Medicine, Health and Life Sciences{{{_:::_}}}Swansea University Medical School - Medicine
document_store_str 0
active_str 0
description In this study the sterol and oxysterol profile of newborn brain from the Dhcr7Δ3-5/T93M mouse model of Smith-Lemli-Opitz syndrome (SLOS) has been investigated. This is a viable mouse model which is compound heterozygous containing one null allele and one T93M mutation on Dhcr7. We find the SLOS mouse has reduced levels of cholesterol and desmosterol and increased levels of 7- and 8-dehydrocholesterol and of 7- and 8-dehydrodesmosterol in brain compared to the wild type. The profile of enzymatically formed oxysterols in the SLOS mouse resembles that in the wild type but the level of 24S-hydroxycholesterol, the dominating cholesterol metabolite, is reduced in a similar proportion to that of cholesterol. A number of oxysterols abundant in the SLOS mouse are probably derived from 7-dehydrocholesterol, however, the mechanism of their formation is unclear.
published_date 2013-12-31T03:27:27Z
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score 11.04748

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