Results 11 to 20 of about 320 (132)

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

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

Oxidation of 7-dehydrocholesterol and desmosterol by human cytochrome P450 46A1[S]

Journal of Lipid Research, 2014
Cytochrome P450 (P450 or CYP) 46A1 is expressed in brain and has been characterized by its ability to oxidize cholesterol to 24S-hydroxycholesterol. In addition, the same enzyme is known to further oxidize 24S-hydroxycholesterol to the 24,25- and 24,27 ...
Sandeep Goyal, Yi Xiao, Ned A. Porter, Libin Xu, F. Peter Guengerich   +4 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

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

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

Journal of Lipid Research, 2014
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.
Ingemar Björkhem, Ulf Diczfalusy, Anita Lövgren-Sandblom, Lena Starck, Monica Jonsson, Keri Tallman, Henrik Schirmer, Lilian Bomme Ousager, Peter J. Crick, Yuqin Wang, William J. Griffiths, F. Peter Guengerich   +11 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

Identification of 8-dehydrocholesterol (cholesta-5,8-dien-3 beta-ol) in patients with Smith-Lemli-Opitz syndrome.

Journal of Lipid Research, 1995
Cholesta-5,8-dien-3 beta-ol (8-dehydrocholesterol) and cholesta-5,7-dien-3 beta-ol (7-dehydrocholesterol) were isolated from the fecal neutral sterol fraction from homozygotes with Smith-Lemli-Opitz syndrome.
A K Batta, G S Tint, S Shefer, D Abuelo, G Salen   +4 more
doaj   +1 more source

Defective conversion of 7-dehydrocholesterol to cholesterol in cultured skin fibroblasts from Smith-Lemli-Opitz syndrome homozygotes

Journal of Lipid Research, 1995
The Smith-Lemli-Opitz syndrome is a common birth defect syndrome characterized biochemically by low plasma cholesterol levels and high concentrations of the cholesterol precursor 7-dehydrocholesterol.
A Honda, G S Tint, G Salen, A K Batta, T S Chen, S Shefer   +5 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