Results 81 to 90 of about 3,608 (158)

New insights into the role of cytochrome P450 reductase (POR) in microsomal redox biology

Acta Pharmaceutica Sinica B, 2012
Cytochrome P450 reductase (POR) is an essential electron transfer protein located on the endoplasmic reticulum of most cell types, and has long been appreciated for its role in cytochrome P450-mediated drug metabolism.
Todd D. Porter
doaj   +1 more source

A membrane defect in the pathogenesis of the Smith-Lemli-Opitz syndrome

Journal of Lipid Research, 2006
The Smith-Lemli-Opitz syndrome (SLOS) is an often lethal birth defect resulting from mutations in the gene responsible for the synthesis of the enzyme 3β-hydroxy-steroid-Δ7-reductase, which catalyzes the reduction of the double bond at carbon 7 on 7 ...
Thomas N. Tulenko, Kathy Boeze-Battaglia, R. Preston Mason, G. Stephen Tint, Robert D. Steiner, William E. Connor, Edward F. Labelle   +6 more
doaj   +1 more source

Effect of DHCR7 on adipocyte differentiation in goats

Animal Biotechnology
Cholesterol is regarded as a signaling molecule in regulating the metabolism and function of fat cells, in which 7-Dehydrocholesterol reductase (DHCR7) is a key enzyme that catalyzes the conversion of 7-dehydrocholesterol to cholesterol, however, the ...
Zhibin Li, Tingting Hu, Ruiwen Li, Jinlan Li, Youli Wang, Yanyan Li, Yaqiu Lin, Yong Wang, Xing Jiani   +8 more
doaj   +1 more source

Metabolic engineering of Saccharomyces cerevisiae for 7-dehydrocholesterol overproduction

Biotechnology for Biofuels, 2018
Background 7-Dehydrocholesterol (7-DHC) has attracted increasing attentions due to its great medical value and the enlarging market demand of its ultraviolet-catalyzed product vitamin D3.
Xiao-Jing Guo, Wen-Hai Xiao, Ying Wang, Ming-Dong Yao, Bo-Xuan Zeng, Hong Liu, Guang-Rong Zhao, Ying-Jin Yuan   +7 more
doaj   +1 more source

Cholic acid increases plasma cholesterol in Smith-Lemli-Opitz syndrome: A pilot study

Molecular Genetics and Metabolism Reports
Background: Smith-Lemli-Opitz syndrome (SLOS) is an inherited disorder of cholesterol biosynthesis associated with congenital malformations, growth delay, intellectual disability and behavior problems.
Ellen R. Elias, Lucas E. Orth, Amy Li, Libin Xu, Sara M. Jones, William B. Rizzo   +5 more
doaj   +1 more source

Oxysterols and Retinal Degeneration in a Rat Model of Smith-Lemli-Opitz Syndrome: Implications for an Improved Therapeutic Intervention

Molecules, 2018
Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive human disease caused by mutations in the gene encoding 7-dehydrocholesterol (7DHC) reductase (DHCR7), resulting in abnormal accumulation of 7DHC and reduced levels of cholesterol in bodily ...
Steven J. Fliesler, Libin Xu
doaj   +1 more source

Post-translational switch of DHCR24 acetylation sustains sterol synthesis and promotes HCC via the 7-ketocholesterol/p62 axis

Cell Reports
Summary: Dysregulated cholesterol synthesis fuels cancer progression, but its precise role in hepatocellular carcinoma (HCC) remains unclear. Here, we identify elevated acetylation of 24-dehydrocholesterol reductase (DHCR24) at Lys254 as a hallmark of ...
Yunfei Zhou, Xinxin Yang, Jianwen Xu, Yidi Wu, Yin Fu, Yihong Dong, Xun Yang, Qiang Fu, Chenxu Guo, Yunjing Hou, Shuyuan Chang, Jun Yan, Ju Ran, Yumeng Wang, Qingxin Zhang, Tomii Ayaka, Lei Yu, Feng Geng, Liuyang Zhao, Xiaoyang Hu, Shuijie Li, Hongxue Meng, Dabin Liu   +22 more
doaj   +1 more source

Clinical Presentation and Diagnosis of Smith-Lemli-Opitz Syndrome: First Case Report from Sudan

Sudan Journal of Medical Sciences
Background: Smith-Lemli-Opitz syndrome (SLOS) is a congenital autosomal recessive disorder characterized by defective cholesterol metabolism, attributable to a deficiency of the enzyme 7-dehydrocholesterol reductase (DHCR7) caused by mutations in the ...
Rayan Khalid, Dalia Omer, Tarig Alsheikh, Imad Fadl-Elmula   +3 more
doaj   +1 more source

Delivery of the 7-dehydrocholesterol reductase gene to the central nervous system using adeno-associated virus vector in a mouse model of Smith-Lemli-Opitz Syndrome. [PDF]

Mol Genet Metab Rep, 2015
Pasta S, Akhile O, Tabron D, Ting F, Shackleton C, Watson G.   +5 more
europepmc   +1 more source

Supporting activities of cognate redox partners for sterol-metabolizing P450 enzymes in Mycobacterium neoaurum. [PDF]

J Biol Chem
Liu Y, Zhao Y, Sun W, Li N, Pan Y, Ma L, Li S.   +6 more
europepmc   +1 more source