Results 21 to 30 of about 7,464,333 (166)

Bile acid synthesis in the Smith-Lemli-Opitz syndrome: effects of dehydrocholesterols on cholesterol 7α-hydroxylase and 27-hydroxylase activities in rat liver

Journal of Lipid Research, 1999
The Smith-Lemli-Opitz syndrome (SLOS) is a congenital birth defect syndrome caused by a deficiency of 3β-hydroxysterol Δ7-reductase, the final enzyme in the cholesterol biosynthetic pathway.
Akira Honda, Gerald Salen, Sarah Shefer, Ashok K. Batta, Megumi Honda, Guorong Xu, G. Stephen Tint, Yasushi Matsuzaki, Junichi Shoda, Naomi Tanaka   +9 more
doaj   +1 more source

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

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

A New Route to 7-Dehydrocholesterol, Provitamin D3 [PDF]

Nature, 1946
IN 1935, Windaus, Lettre and Schenck1 described the preparation from cholesterol (I) of 7-dehydrocholesterol (III), which on irradiation gave a highly antirachitic product (vitamin D3), later2 shown to be identical with the naturally occurring vitamin D isolated from tunny liver oil.
H. B. HENBEST, E. R. H. JONES, A. E. BIDE, R. W. PEEVERS, P. A. WILKINSON   +4 more
openaire   +2 more sources

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

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

7-Dehydrocholesterol is an endogenous suppressor of ferroptosis [PDF]

, 2021
Abstract Ferroptosis is a form of cell death that has received considerable attention not only as a means to eradicate defined tumour entities but also because it provides unforeseen insights into the metabolic adaptation exploited by tumours to counteract phospholipid oxidation.
Jose Pedro Friedmann Angeli, Florencio Porto Freitas, Palina Nepachalovich, Lohans Puentes, Omkar Zilka, Alex Inague, Svenja Lorenz, Viktoria Kunz, Helene Nehring, Thamara Nishida Xavier da Silva, Zhiyi Chen, Sebastian Doll, Werner Schmitz, Peter Imming, Sayuri Miyamoto, Judith Klein-Seetharaman, Lokender Kumar, Thiago Cardoso Genaro-Mattos, Karoly Mirnics, Svenja Meierjohann, Matthias Kroiss, Isabel Weigand, Kurt Bommert, Ralf Bargou, Ana Garcia-Saez, Derek Pratt, Maria Fedorova, Ann Wehmann, Aline Horling, Georg Bornkamm, Marcus Conrad   +30 more
openaire   +1 more source

UVB-Induced Conversion of 7-Dehydrocholesterol to 1α,25-Dihydroxyvitamin D3 in an In Vitro Human Skin Equivalent Model [PDF]

, 2001
We have previously shown that keratinocytes in vitro can convert biologically inactive vitamin D3 to the hormone calcitriol (1α,25-dihydroxyvitamin D3).
Lehmann, Bodo, Pietzsch, Jens, Meurer, Michael, Knuschke, Peter, Genehr, Thurid   +4 more
core   +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