Results 41 to 50 of about 23,617 (228)

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

Cell-specific discrimination of desmosterol and desmosterol mimetics confers selective regulation of LXR and SREBP in macrophages. [PDF]

, 2018
Activation of liver X receptors (LXRs) with synthetic agonists promotes reverse cholesterol transport and protects against atherosclerosis in mouse models.
Bahadorani, John, Edillor, Chantle R, Glass, Christopher K, Grossman, Tamar R, Henke, Adam, McDonald, Jeffrey G, Muse, Evan D, Ozeki, Katherine A, Roland, Jason T, Seidman, Jason S, Spann, Nathanael J, Tao, Jenhan, Thompson, Bonne M, Tremblay, Matthew S, Troutman, Ty D, Tsimikas, Sotirios, Yu, Shan   +16 more
core   +1 more source

A GC polymorphism associated with serum 25-hydroxyvitamin D level is a risk factor for hip fracture in Japanese patients with rheumatoid arthritis: 10-year follow-up of the Institute of Rheumatology, Rheumatoid Arthritis cohort study [PDF]

, 2014
INTRODUCTION: Vitamin D deficiency has been reported to be common in patients with rheumatoid arthritis (RA) who have a higher prevalence of osteoporosis and hip fracture than healthy individuals.
FURUYA,　Takefumi, IKARI,　Katsunori, MOMOHARA,　Shigeki, TANIGUCHI,　Atsuo, TOYAMA,　Yoshiaki, YAMANAKA,　Hisashi, YOSHIDA,　Shinji   +6 more
core   +6 more sources

Down-regulation of cholesterol biosynthesis in sitosterolemia: diminished activities of acetoacetyl-CoA thiolase, 3-hydroxy-3-methylglutaryl-CoA synthase, reductase, squalene synthase, and 7-dehydrocholesterol Δ7-reductase in liver and mononuclear leukocytes

Journal of Lipid Research, 1998
Sitosterolemia is a recessively inherited disorder characterized by abnormally increased plasma and tissue plant sterol concentrations. Patients have markedly reduced whole body cholesterol biosynthesis associated with suppressed hepatic, ileal, and ...
Akira Honda, Gerald Salen, Lien B. Nguyen, G. Stephen Tint, Ashok K. Batta, Sarah Shefer   +5 more
doaj   +1 more source

DHCR7 as a Prognostic and Immunological Biomarker in Human Pan-Cancer: A Comprehensive Evaluation. [PDF]

Cancer Rep (Hoboken)
ABSTRACT Background The 7‐Dehydrocholesterol reductase (DHCR7), a critical enzyme catalyzing the final step of the cholesterol biosynthesis pathway, has gained attention for its potential role in tumorigenesis. This study systematically investigated the association between DHCR7 expression and oncogenic processes across multiple cancer types.
Wu X, Zheng W, Wang L, Lin D, Wu Z.
europepmc   +2 more sources

Regulation of rat hepatic 3β-hydroxysterol Δ7-reductase: substrate specificity, competitive and non-competitive inhibition, and phosphorylation/dephosphorylation

Journal of Lipid Research, 1998
The mechanism for the catalytic reduction of the double bond at C-7,8 in 7-dehydrocholesterol by 3β-hydroxysterol Δ7-reductase was investigated by testing structurally related sterols as substrates and potential inhibitors. The hepatic smooth endoplasmic
S. Shefer, G. Salen, A. Honda, A.K. Batta, L.B. Nguyen, G.S. Tint, Y.A. Ioannou, R. Desnick   +7 more
doaj   +1 more source

Sterol profiles in plasma and erythrocyte membranes in patients with Smith-Lemli-Opitz syndrome: a six-year experience [PDF]

, 2011
Background: This study reports our experience over the last six years in the diagnosis of Smith-Lemli-Opitz syndrome and other inborn errors of cholesterol biosynthesis.
A. Nappi, DELLO RUSSO, ANTONIO, G. Corso, GELZO, MONICA, P. Pianese, R. Barone, S. Clericuzio   +6 more
core   +1 more source

Identification and characterization of prescription drugs that change levels of 7-dehydrocholesterol and desmosterol

Journal of Lipid Research, 2018
Regulating blood cholesterol (Chol) levels by pharmacotherapy has successfully improved cardiovascular health. There is growing interest in the role of Chol precursors in the treatment of diseases.
Phillip A. Wages, Hye-Young H. Kim, Zeljka Korade, Ned A. Porter   +3 more
doaj   +1 more source