Description
8-dehydro Cholesterol (8-DHC) is an isomer of the cholesterol precursor 7-DHC . It accumulates in tissues and plasma in patients with Smith-Lemli-Opitz syndrome (SLOS), a disorder characterized by a mutation in
DHCR7, the gene encoding 3β-hydroxysterol-Δ
7-reductase, and decreased cholesterol levels in bodily tissues and fluids, as well as microcephaly, intellectual disability, and distinctive dysmorphic features. 8-DHC levels are increased in serum in a rat model of SLOS induced by the DHCR7 inhibitor AY 9944 .
Uses
8-Dehydrocholesterol is an intermediate in the synthesis of Zymostenol, which is the derivative of Zymosterol (Z701520), an sterol intermediate in the biosynthesis of cholesterol (C432501).
Definition
ChEBI: 8-Dehydrocholesterol is a steroid.
IC 50
Human Endogenous Metabolite
References
[1] C WOLF. Changes in serum sterols of rats treated with 7-dehydrocholesterol-delta 7-reductase inhibitors: comparison to levels in humans with Smith-Lemli-Opitz syndrome.[J]. Journal of Lipid Research, 1996, 37 6: 1325-1333.
[2] De Souza, F.I., Zumiotti, A.V., and Da Silva, C.F. Neuregulins 1-α and 1-β on the regeneration the peripheral nerves[J]. Acta Ortop Bras.
[3] WILLIAM J. GRIFFITHS . Sterols and oxysterols in plasma from Smith-Lemli-Opitz syndrome patients[J]. Journal of Steroid Biochemistry and Molecular Biology, 2017, 169: Pages 77-87. DOI:
10.1016/j.jsbmb.2016.03.018
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