Results 111 to 120 of about 16,041 (210)

A point mutation in the bile acid biosynthetic enzyme sterol 27-hydroxylase in a family with cerebrotendinous xanthomatosis.

Journal of Lipid Research, 1994
Cerebrotendinous xanthomatosis (CTX) is a rare familial disorder characterized by progressive neurological dysfunction, atherosclerosis, and xanthomas with sterol storage in the nervous system, vessels, and tendons.
N. Nakashima, Y. Sakai, H. Sakai, T. Yanase, M. Haji, F. Umeda, S. Koga, T. Hoshita, H. Nawata   +8 more
doaj   +1 more source

Current trends in oxysterol research [PDF]

, 2016
In this short review we provide a synopsis of recent developments in oxysterol research highlighting topics of current interest to the community. These include the involvement of oxysterols in neuronal development and survival, their participation in the
Alvelius, Alwena H. Morgan, Andersson, Arnoldi, Bauman, Blanc, Clayton, Crick, de Medina, Diczfalusy, Dietschy, Eylan Yutuc, Fakheri, Gill, Goyal, Hannedouche, Heverin, Horton, Hu, Iuliano, Iuliano, Janowski, Jansen, Javitt, Jonas Abdel-Khalik, Klinke, Leonarduzzi, Liu, Lütjohann, Maarup, Meaney, Meljon, Murphy, Nelson, Ogundare, Porter, Radhakrishnan, Reboldi, Russell, Sacchetti, Santori, Schroepfer, Shackleton, Song, Soroosh, Theofilopoulos, Theofilopoulos, Thomas Hearn, Tint, Umetani, Wang, Wang, William J. Griffiths, Wu, Yuqin Wang   +54 more
core   +1 more source

Hexose-6-phosphate dehydrogenase modulates the effect of inhibitors and alternative substrates of 11[beta]-hydroxysteroid dehydrogenase 1 [PDF]

, 2008
Intracellular glucocorticoid reactivation is catalyzed by 11[beta]-hydroxysteroid dehydrogenase 1 (11[beta]-HSD1), which functions predominantly as a reductase in cells expressing hexose-6-phosphate dehydrogenase (H6PDH).
Alex Odermatt, Charlie Chandsawangbhuwana, Lyubomir G. Nashev, Michael E. Baker, Zoltá   +4 more
core   +1 more source

25R,26-Hydroxycholesterol revisited: synthesis, metabolism, and biologic roles

Journal of Lipid Research, 2002
The CYP27 gene is expressed in arterial endothelium, macrophages, and other tissues. The gene product generates sterol intermediates that function as ligands for nuclear receptors prior to their transport to the liver for metabolism, mostly to bile acids.
Norman B. Javitt
doaj   +1 more source

Perturbed cholesterol and vesicular trafficking associated with dengue blocking in Wolbachia-infected Aedes aegypti cells [PDF]

, 2017
Wolbachia are intracellular maternally inherited bacteria that can spread through insect populations and block virus transmission by mosquitoes, providing an important approach to dengue control.
Ant, Thomas H., Charles, Philip D., Dowle, Adam A., Geoghegan, Vincent, Hester, Svenja, Larson, Tony, Rainey, Stephanie M., Sinkins, Steven P., Stainton, Kirsty, Thomas, Benjamin   +9 more
core   +3 more sources

Structure and functions of human oxysterol 7α-hydroxylase cDNAs and gene CYP7B1

Journal of Lipid Research, 1999
Oxysterol 7α-hydroxylase has broad substrate specificity for sterol metabolites and may be involved in many metabolic processes including bile acid synthesis and neurosteroid metabolism.
Zhengliang Wu, K.O. Martin, N.B. Javitt, J.Y.L. Chiang   +3 more
doaj   +1 more source

Expression of key enzymes in bile acid biosynthesis during development: CYP7B1-mediated activities show tissue-specific differences

Journal of Lipid Research, 2002
The developmental variation of cytochrome P450 (CYP)7A1, CYP7B1, CYP27A1, and 3β-hydroxy-Δ5-C27-steroid dehydrogenase, key enzymes in bile acid biosynthesis, were investigated in pigs of different ages.
Maria Norlin
doaj   +1 more source

Human Sterol 27-Hydroxylase (CYP27) Overexpressor Transgenic Mouse Model [PDF]

Journal of Biological Chemistry, 2002
Karen Meir, Daniel Kitsberg, Irit Alkalay, Fanny Szafer, Haim Rosen, Shoshanna Shpitzen, Liat Ben Avi, Bart Staels, Catherine Fievet, Vardiella Meiner, Ingemar Bjo¨rkhem, Eran Leitersdorf   +11 more
openaire   +1 more source

Biosynthesis of the regulatory oxysterol, 5-cholesten-3β,25-diol 3-sulfate, in hepatocytes

Journal of Lipid Research, 2007
Cellular cholesterol homeostasis is maintained through coordinated regulation of cholesterol synthesis, degradation, and secretion. Nuclear receptors for oxygenated cholesterol derivatives (oxysterols) are known to play key roles in the regulation of ...
Xiaobo Li, William M. Pandak, Sandra K. Erickson, Yongjie Ma, Lianhua Yin, Phillip Hylemon, Shunlin Ren   +6 more
doaj   +1 more source

Cerebrotendinous xanthomatosis-a case report with novel compound heterozygous mutation of CYP27A1 gene

Dermatologica Sinica, 2018
Cerebrotendinous xanthomatosis (CTX), a rare autosomal recessive lipid storage disorder which is caused by mutations in gene CYP27A1 leads to deficiency of enzyme sterol 27-hydroxylase.
Jeng Yuan, Pi-Shan Sung, Julia Yu-Yun Lee, Sheau-Chiou Chao   +3 more
doaj   +1 more source