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On the formation of 7-ketocholesterol from 7-dehydrocholesterol in patients with CTX and SLO

I. Bjorkhem+U., William Griffiths Orcid Logo, Yuqin Wang Orcid Logo

The Journal of Lipid Research

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

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DOI (Published version): 10.1194/jlr.P048603

Abstract

A new mechanism for formation of 7-ketocholesterol was recently described involving cytochrome P-450 (CYP) 7A1- catalyzed conversion of 7-dehydrocholesterol into 7-ketocholesterol with cholesterol-7,8-epoxide as a side product (Shinkyo R et al. J. Biol.Chem. 2011; 286:33021-33028). Some patients wit...

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Published in: The Journal of Lipid Research
Published: 2014
URI: https://cronfa.swan.ac.uk/Record/cronfa17919
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first_indexed 2014-05-02T01:30:02Z
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fullrecord <?xml version="1.0"?><rfc1807><datestamp>2015-11-23T15:38:30.4355184</datestamp><bib-version>v2</bib-version><id>17919</id><entry>2014-05-01</entry><title>On the formation of 7-ketocholesterol from 7-dehydrocholesterol in patients with CTX and SLO</title><swanseaauthors><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><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></swanseaauthors><date>2014-05-01</date><deptcode>BMS</deptcode><abstract>A new mechanism for formation of 7-ketocholesterol was recently described involving cytochrome P-450 (CYP) 7A1- catalyzed conversion of 7-dehydrocholesterol into 7-ketocholesterol with cholesterol-7,8-epoxide as a side product (Shinkyo R et al. J. Biol.Chem. 2011; 286:33021-33028). Some patients with Cerebrotendinous Xanthomatosis (CTX) and all patients with Smith Lemli Opitz syndrome (SLO) have markedly increased levels of 7-dehydrocholesterol in plasma and tissues. In addition the former patients havemarkedly up-regulated CYP7A1. We hypothesized that these patients may produce 7-ketocholesterol from 7-dehydrocholesterol with formation of cholesterol-7,8-epoxide as a side product. In accord with this hypothesis two patients with CTX were found to have increased levels of 7-ketocholesterol and 7-dehydrocholesterol as well as significant level of cholesterol-7,8-epoxide. The latter steroid was not detectable in plasma from healthy volunteers. Down-regulation of CYP7A1 activity by treatment with chenodeoxycholic acid reduced the levels of 7-ketocholesterol in parallel with decreased levels of 7-dehydrocholesterol and cholesterol-7,8-epoxide. Three patients with SLO were found to have markedly elevated levels of 7-ketocholesterol as well as high levels of cholesterol-7,8-epoxide. The results support the hypothesis that 7-dehydrocholesterol is a precursor to 7-ketocholesterol in SLO and some patients with CTX.</abstract><type>Journal Article</type><journal>The Journal of Lipid Research</journal><publisher/><keywords/><publishedDay>31</publishedDay><publishedMonth>12</publishedMonth><publishedYear>2014</publishedYear><publishedDate>2014-12-31</publishedDate><doi>10.1194/jlr.P048603</doi><url/><notes></notes><college>COLLEGE NANME</college><department>Biomedical Sciences</department><CollegeCode>COLLEGE CODE</CollegeCode><DepartmentCode>BMS</DepartmentCode><institution>Swansea University</institution><apcterm/><lastEdited>2015-11-23T15:38:30.4355184</lastEdited><Created>2014-05-01T14:13:11.8737991</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>I.</firstname><surname>Bjorkhem+U.</surname><order>1</order></author><author><firstname>William</firstname><surname>Griffiths</surname><orcid>0000-0002-4129-6616</orcid><order>2</order></author><author><firstname>Yuqin</firstname><surname>Wang</surname><orcid>0000-0002-3063-3066</orcid><order>3</order></author></authors><documents/><OutputDurs/></rfc1807>
spelling 2015-11-23T15:38:30.4355184 v2 17919 2014-05-01 On the formation of 7-ketocholesterol from 7-dehydrocholesterol in patients with CTX and SLO 3316b1d1b524be1831790933eed1c26e 0000-0002-4129-6616 William Griffiths William Griffiths true false c92729b58622f9fdf6a0e7d8f4ce5081 0000-0002-3063-3066 Yuqin Wang Yuqin Wang true false 2014-05-01 BMS A new mechanism for formation of 7-ketocholesterol was recently described involving cytochrome P-450 (CYP) 7A1- catalyzed conversion of 7-dehydrocholesterol into 7-ketocholesterol with cholesterol-7,8-epoxide as a side product (Shinkyo R et al. J. Biol.Chem. 2011; 286:33021-33028). Some patients with Cerebrotendinous Xanthomatosis (CTX) and all patients with Smith Lemli Opitz syndrome (SLO) have markedly increased levels of 7-dehydrocholesterol in plasma and tissues. In addition the former patients havemarkedly up-regulated CYP7A1. We hypothesized that these patients may produce 7-ketocholesterol from 7-dehydrocholesterol with formation of cholesterol-7,8-epoxide as a side product. In accord with this hypothesis two patients with CTX were found to have increased levels of 7-ketocholesterol and 7-dehydrocholesterol as well as significant level of cholesterol-7,8-epoxide. The latter steroid was not detectable in plasma from healthy volunteers. Down-regulation of CYP7A1 activity by treatment with chenodeoxycholic acid reduced the levels of 7-ketocholesterol in parallel with decreased levels of 7-dehydrocholesterol and cholesterol-7,8-epoxide. Three patients with SLO were found to have markedly elevated levels of 7-ketocholesterol as well as high levels of cholesterol-7,8-epoxide. The results support the hypothesis that 7-dehydrocholesterol is a precursor to 7-ketocholesterol in SLO and some patients with CTX. Journal Article The Journal of Lipid Research 31 12 2014 2014-12-31 10.1194/jlr.P048603 COLLEGE NANME Biomedical Sciences COLLEGE CODE BMS Swansea University 2015-11-23T15:38:30.4355184 2014-05-01T14:13:11.8737991 Faculty of Medicine, Health and Life Sciences Swansea University Medical School - Medicine I. Bjorkhem+U. 1 William Griffiths 0000-0002-4129-6616 2 Yuqin Wang 0000-0002-3063-3066 3
title On the formation of 7-ketocholesterol from 7-dehydrocholesterol in patients with CTX and SLO
spellingShingle On the formation of 7-ketocholesterol from 7-dehydrocholesterol in patients with CTX and SLO
William Griffiths
Yuqin Wang
title_short On the formation of 7-ketocholesterol from 7-dehydrocholesterol in patients with CTX and SLO
title_full On the formation of 7-ketocholesterol from 7-dehydrocholesterol in patients with CTX and SLO
title_fullStr On the formation of 7-ketocholesterol from 7-dehydrocholesterol in patients with CTX and SLO
title_full_unstemmed On the formation of 7-ketocholesterol from 7-dehydrocholesterol in patients with CTX and SLO
title_sort On the formation of 7-ketocholesterol from 7-dehydrocholesterol in patients with CTX and SLO
author_id_str_mv 3316b1d1b524be1831790933eed1c26e
c92729b58622f9fdf6a0e7d8f4ce5081
author_id_fullname_str_mv 3316b1d1b524be1831790933eed1c26e_***_William Griffiths
c92729b58622f9fdf6a0e7d8f4ce5081_***_Yuqin Wang
author William Griffiths
Yuqin Wang
author2 I. Bjorkhem+U.
William Griffiths
Yuqin Wang
format Journal article
container_title The Journal of Lipid Research
publishDate 2014
institution Swansea University
doi_str_mv 10.1194/jlr.P048603
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 A new mechanism for formation of 7-ketocholesterol was recently described involving cytochrome P-450 (CYP) 7A1- catalyzed conversion of 7-dehydrocholesterol into 7-ketocholesterol with cholesterol-7,8-epoxide as a side product (Shinkyo R et al. J. Biol.Chem. 2011; 286:33021-33028). Some patients with Cerebrotendinous Xanthomatosis (CTX) and all patients with Smith Lemli Opitz syndrome (SLO) have markedly increased levels of 7-dehydrocholesterol in plasma and tissues. In addition the former patients havemarkedly up-regulated CYP7A1. We hypothesized that these patients may produce 7-ketocholesterol from 7-dehydrocholesterol with formation of cholesterol-7,8-epoxide as a side product. In accord with this hypothesis two patients with CTX were found to have increased levels of 7-ketocholesterol and 7-dehydrocholesterol as well as significant level of cholesterol-7,8-epoxide. The latter steroid was not detectable in plasma from healthy volunteers. Down-regulation of CYP7A1 activity by treatment with chenodeoxycholic acid reduced the levels of 7-ketocholesterol in parallel with decreased levels of 7-dehydrocholesterol and cholesterol-7,8-epoxide. Three patients with SLO were found to have markedly elevated levels of 7-ketocholesterol as well as high levels of cholesterol-7,8-epoxide. The results support the hypothesis that 7-dehydrocholesterol is a precursor to 7-ketocholesterol in SLO and some patients with CTX.
published_date 2014-12-31T03:20:52Z
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score 11.021648

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