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

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

doi:10.1016/j.bcp.2013.03.003

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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

, Cedric H.L Shackleton, William Griffiths

Biochemical Pharmacology

Swansea University Authors: Yuqin Wang , William Griffiths

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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
Online Access:	Check full text
URI:	https://cronfa.swan.ac.uk/Record/cronfa14485
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first_indexed	2013-07-23T12:12:18Z
last_indexed	2018-02-09T04:45:53Z
id	cronfa14485
recordtype	SURis
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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 BMS 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 Biomedical Sciences COLLEGE CODE BMS 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
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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	2013-12-31T03:16:35Z
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score	10.999547

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

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