Results 11 to 20 of about 16,041 (210)

Heterogeneous expression of cholesterol 7 alpha-hydroxylase and sterol 27-hydroxylase genes in the rat liver lobulus. [PDF]

Journal of Clinical Investigation, 1995
We investigated the lobular localization and molecular level of expression of cholesterol 7 alpha-hydroxylase and sterol 27-hydroxylase, two key enzymes in bile acid synthesis, in isolated periportal and pericentral hepatocytes and by in situ hybridization of rat liver.
Twisk, J., Hoekman, M.F.M., Mager, W.H., Moorman, A.F.M., Boer, P.A.J. de, Scheja, L., Princen, H.M.G., Gebhardt, R.   +7 more
openaire   +5 more sources

Four novel mutations of sterol 27-hydroxylase gene in Italian patients with cerebrotendinous xanthomatosis

Journal of Lipid Research, 1997
We report the characterization of eight mutations of sterol 27-hydroxylase gene (CYP27) in five Italian patients with cerebrotendinous xanthomatosis, who were found to be compound heterozygotes.
R Garuti, M A Croce, R Tiozzo, M T Dotti, A Federico, S Bertolini, S Calandra   +6 more
doaj   +6 more sources

Sterol 27-Hydroxylase Acts on 7-Ketocholesterol in Human Atherosclerotic Lesions and Macrophages in Culture [PDF]

Journal of Biological Chemistry, 2000
27-Hydroxycholesterol (27OH) is the major oxysterol in human atherosclerotic lesions, followed by 7-ketocholesterol (7K). Whereas 7K probably originates nonenzymically, 27OH arises by the action of sterol 27-hydroxylase, a cytochrome P450 enzyme expressed at particularly high levels in the macrophage and proposed to represent an important pathway by ...
Brown, Andrew J., Watts, Gerald F., Burnett, John R., Dean, Roger T., Jessup, Wendy K.   +4 more
openaire   +5 more sources

Expression of Sterol 27-Hydroxylase (CYP27A1) Enhances Cholesterol Efflux [PDF]

Journal of Biological Chemistry, 2003
Cholesterol efflux from CHOP cells transfected with sterol 27-hydroxylase (CYP27A1) was compared with non-transfected and mock-transfected cells. Transfection caused expression of CYP27A1, formation of 27-hydroxycholesterol, and inhibition of cholesterol biosynthesis. Transfection enhanced cholesterol efflux to apolipoprotein A-I or human plasma by 2-3-
Genevieve, Escher, Zygmunt, Krozowski, Kevin D, Croft, Dmitri, Sviridov   +3 more
openaire   +2 more sources

Localization of sterols and oxysterols in mouse brain reveals distinct spatial cholesterol metabolism [PDF]

, 2020
Dysregulated cholesterol metabolism is implicated in a number of neurological disorders. Many sterols, including cholesterol and its precursors and metabolites, are biologically active and important for proper brain function. However, spatial cholesterol
Angelini, Roberto, Baumert, Mark, Clench, Malcolm, Griffiths, William J., Howell, Owain W., Mast, Natalia, Newton, Jillian, Pikuleva, Irina, Skibinski, David O.F., Wang, Yuqin, Yutuc, Eylan   +10 more
core   +2 more sources

Cerebrotendinous xanthomatosis: a family study of sterol 27-hydroxylase mutations and pharmacotherapy [PDF]

QJM, 1996
We examined the phenotypic characteristics, molecular genetics and optimal pharmacological treatment of cerebrotendinous xanthomatosis (CTX) in an English family with combined hyperlipidaemia. The proband presented in adulthood with classical clinical characteristics of CTX, a greater than tenfold elevation in plasma cholestanol and combined ...
G F, Watts, W D, Mitchell, J J, Bending, A, Reshef, E, Leitersdorf   +4 more
openaire   +2 more sources

Exon skipping in the sterol 27-hydroxylase gene leads to cerebrotendinous xanthomatosis [PDF]

Human Genetics, 1997
We report a new mutation in the sterol 27-hydroxylase (CYP 27) gene in a Dutch family with cerebrotendinous xanthomatosis: a G-->A transition in the splice donor site in intron 4. This mutation leads to skipping of exon 4, resulting in a loss of 66 amino acids in the CYP 27 enzyme molecule.
Verrips, A., Steenbergen-Spanjers, G.C.H., Luyten, J.A.F.M., Wevers, R.A., Wokke, J.H.J., Gabreëls, F.J.M., Wolthers, B.G., Heuvel, L.P.W.J. van den   +7 more
openaire   +6 more sources

Elimination of Cholesterol in Macrophages and Endothelial Cells by the Sterol 27-Hydroxylase Mechanism [PDF]

Journal of Biological Chemistry, 1997
Amir Babiker, Olof Andersson, Erik Lund, Rui-Juan Xiu, Samir Deeb, Ayeleth Reshef, Eran Leitersdorf, Ulf Diczfalusy, Ingemar Björkhem   +8 more
openaire   +3 more sources

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

Genetic obesity increases pancreatic expression of mitochondrial proteins which regulate cholesterol efflux in BRIN-BD11 insulinoma cells [PDF]

, 2019
Pancreatic β-cells are sensitive to fluctuations in cholesterol content, which can damage the insulin secretion pathway, contributing to the aetiology of type 2 diabetes mellitus.
Caridis, Anna Maria, Dolan, Sharron, Graham, Annette, Lightbody, Richard J., Tarlton, Jamie M.R.   +4 more
core   +1 more source