Journal article 1217 views
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
Biochemical Pharmacology
Swansea University Authors:
Yuqin Wang , William Griffiths
Full text not available from this repository: check for access using links below.
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...
Published in: | Biochemical Pharmacology |
---|---|
ISSN: | 0006-2952 |
Published: |
2013
|
Online Access: |
Check full text
|
URI: | https://cronfa.swan.ac.uk/Record/cronfa14485 |
Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
first_indexed |
2013-07-23T12:12:18Z |
---|---|
last_indexed |
2018-02-09T04:45:53Z |
id |
cronfa14485 |
recordtype |
SURis |
fullrecord |
<?xml version="1.0"?><rfc1807><datestamp>2013-08-01T08:01:09.5847727</datestamp><bib-version>v2</bib-version><id>14485</id><entry>2013-03-26</entry><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</title><swanseaauthors><author><sid>c92729b58622f9fdf6a0e7d8f4ce5081</sid><ORCID>0000-0002-3063-3066</ORCID><firstname>Yuqin</firstname><surname>Wang</surname><name>Yuqin Wang</name><active>true</active><ethesisStudent>false</ethesisStudent></author><author><sid>3316b1d1b524be1831790933eed1c26e</sid><ORCID>0000-0002-4129-6616</ORCID><firstname>William</firstname><surname>Griffiths</surname><name>William Griffiths</name><active>true</active><ethesisStudent>false</ethesisStudent></author></swanseaauthors><date>2013-03-26</date><deptcode>BMS</deptcode><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 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.</abstract><type>Journal Article</type><journal>Biochemical Pharmacology</journal><volume></volume><journalNumber></journalNumber><paginationStart/><paginationEnd/><publisher/><placeOfPublication/><issnPrint>0006-2952</issnPrint><issnElectronic/><keywords/><publishedDay>31</publishedDay><publishedMonth>12</publishedMonth><publishedYear>2013</publishedYear><publishedDate>2013-12-31</publishedDate><doi>10.1016/j.bcp.2013.03.003</doi><url/><notes/><college>COLLEGE NANME</college><department>Biomedical Sciences</department><CollegeCode>COLLEGE CODE</CollegeCode><DepartmentCode>BMS</DepartmentCode><institution>Swansea University</institution><apcterm/><lastEdited>2013-08-01T08:01:09.5847727</lastEdited><Created>2013-03-26T13:52:03.4937879</Created><path><level id="1">Faculty of Medicine, Health and Life Sciences</level><level id="2">Swansea University Medical School - Medicine</level></path><authors><author><firstname>Anna</firstname><surname>Meljon</surname><order>1</order></author><author><firstname>Gordon L</firstname><surname>Watson</surname><order>2</order></author><author><firstname>Yuqin</firstname><surname>Wang</surname><orcid>0000-0002-3063-3066</orcid><order>3</order></author><author><firstname>Cedric H.L</firstname><surname>Shackleton</surname><order>4</order></author><author><firstname>William</firstname><surname>Griffiths</surname><orcid>0000-0002-4129-6616</orcid><order>5</order></author></authors><documents/><OutputDurs/></rfc1807> |
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 |
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 |
2013-12-31T03:16:35Z |
_version_ |
1763750335149506560 |
score |
11.021648 |