Results 21 to 30 of about 3,608 (158)

Negative regulation of Hedgehog signaling by the cholesterogenic enzyme 7-dehydrocholesterol reductase [PDF]

Development, 2006
Cholesterol regulates Hedgehog (Hh) signaling during early vertebrate development. Smith-Lemli-Opitz syndrome (SLOS) is caused by defects in 7-dehydrocholesterol reductase (DHCR7), an enzyme catalyzing the final step of cholesterol biosynthesis. Many developmental malformations attributed to SLOS occur in tissues and organs where Hh signaling is ...
Tetsuya, Koide, Tadayoshi, Hayata, Ken W Y, Cho   +2 more
openaire   +2 more sources

The terminal enzymes of cholesterol synthesis, DHCR24 and DHCR7, interact physically and functionally[S]

Journal of Lipid Research, 2015
Cholesterol is essential to human health, and its levels are tightly regulated by a balance of synthesis, uptake, and efflux. Cholesterol synthesis requires the actions of more than twenty enzymes to reach the final product, through two alternate ...
Winnie Luu, Gene Hart-Smith, Laura J. Sharpe, Andrew J. Brown   +3 more
doaj   +1 more source

A dual role for 7-dehydrocholesterol reductase in regulating Hedgehog signalling? [PDF]

Development, 2006
In a recent paper published in Development , Koide et al. provide evidence for a negative regulatory action of 7-dehydrocholesterol reductase (DCHR7) on the Hedgehog pathway ([Koide et al., 2006][1]).
Bijlsma, Maarten F., Peppelenbosch, Maikel P., Spek, C. Arnold   +2 more
openaire   +1 more source

7-Dehydrocholesterol down-regulates cholesterol biosynthesis in cultured Smith-Lemli-Opitz syndrome skin fibroblasts

Journal of Lipid Research, 1998
The Smith-Lemli-Opitz syndrome (SLOS) is a common birth defect–mental retardation syndrome caused by a defect in the enzyme that reduces 7-dehydrocholesterol to cholesterol.
Megumi Honda, G.S. Tint, Akira Honda, Lien B. Nguyen, Thomas S. Chen, Sarah Shefer   +5 more
doaj   +1 more source

Vulnerability of DHCR7+/− mutation carriers to aripiprazole and trazodone exposure

Journal of Lipid Research, 2017
Smith-Lemli-Opitz syndrome is a recessive disorder caused by mutations in 7-dehydrocholesterol reductase (DHCR)7 with a heterozygous (HET) carrier frequency of 1–3%.
Zeljka Korade, Thiago C. Genaro-Mattos, Keri A. Tallman, Wei Liu, Krassimira A. Garbett, Katalin Koczok, Istvan Balogh, Karoly Mirnics, Ned A. Porter   +8 more
doaj   +1 more source

Desmosterol and 7-dehydrocholesterol concentrations in post mortem brains of depressed people: The role of trazodone

Translational Psychiatry, 2022
Major depressive disorder (MDD) is a common, disabling, and heterogeneous condition that responds unpredictably to current treatments. We previously showed an association between depressive symptoms and plasma concentrations of two cholesterol precursors,
Basar Cenik, Jayme M. Palka, Bonne M. Thompson, Jeffrey G. McDonald, Carol A. Tamminga, Can Cenik, E. Sherwood Brown   +6 more
doaj   +1 more source

Changes in serum sterols of rats treated with 7-dehydrocholesterol-delta 7-reductase inhibitors: comparison to levels in humans with Smith-Lemli-Opitz syndrome.

Journal of Lipid Research, 1996
The impaired conversion of 7-dehydrocholesterol to cholesterol, as a result of a permanent inhibition of the activity of 7-dehydrocholesterol-delta 7-reductase, has been reported in the Smith-Lemli-Opitz (SLO) syndrome (1, 2).
C Wolf, F Chevy, J Pham, M Kolf-Clauw, D Citadelle, N Mulliez, C Roux   +6 more
doaj   +1 more source

Abnormal cholesterol biosynthesis in the Smith-Lemli-Opitz syndrome.

Journal of Lipid Research, 1996
The Smith-Lemli-Opitz syndrome is caused by an inherited defect in 7-dehydrocholesterol-delta7-reductase, the enzyme that catalyzes the last reaction in cholesterol biosynthesis, the conversion of 7-dehydrocholesterol to cholesterol.
G Salen, S Shefer, A K Batta, G S Tint, G Xu, A Honda, M Irons, E R Elias   +7 more
doaj   +1 more source

Oxidized derivatives of 7-dehydrocholesterol induce growth retardation in cultured rat embryos: a model for antenatal growth retardation in the Smith-Lemli-Opitz syndrome

Journal of Lipid Research, 1999
7-Dehydrocholesterol accumulates in fetuses affected by the Smith-Lemli-Opitz syndrome as a result of a deficit in the ultimate step of cholesterol synthesis catalyzed by Δ7 reductase.
Wassila Gaoua, Françoise Chevy, Charles Roux, Claude Wolf   +3 more
doaj   +1 more source

Effect of a new inhibitor of cholesterol biosynthesis (AY 9944) on serum and tissue sterols in the rat

Journal of Lipid Research, 1966
AY 9944 is a novel inhibitor of cholesterol biosynthesis which appears to act on the Δ7-reductase enzyme, interfering with the conversion of 7-dehydrocholesterol to cholesterol.
L. Horlick
doaj   +1 more source