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

Permanent lenke
https://hdl.handle.net/10037/25323
DOI
https://doi.org/10.1194/jlr.P048603
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article.pdf (901.0Kb)
Publisert versjon (PDF)
Dato
2014-04-25
Type
Journal article
Tidsskriftartikkel
Peer reviewed

Forfatter
Björkhem, Ingemar; Diczfalusy, Ulf; Lövgren-Sandblom, Anita; Starck, Lena; Jonsson, Monica; Tallman, Keri; Schirmer, Henrik; Ousager, Lillian Bomme; Crick, Peter J; Wang, Yuqing; Griffiths, William J; Guengerich, F.Peter
Sammendrag
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. 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 have markedly upregulated 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 a signifi cant level of cholesterol-7,8-epoxide. The latter steroid was not detectable in plasma from healthy volunteers. Downregulation 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.
Forlag
Elsevier
Sitering
Björkhem I, Diczfalusy, Lövgren-Sandblom, Starck, Jonsson, Tallman, Schirmer H, Ousager, Crick, Wang Y, Griffiths, Guengerich. On the formation of 7-ketocholesterol from 7-dehydrocholesterol in patients with CTX and SLO. Journal of Lipid Research. 2014;55(6):1165-1172
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Samlinger
  • Artikler, rapporter og annet (klinisk medisin) [1974]
Copyright 2014 The American Society for Biochemistry and Molecular Biology, Inc

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