Results 31 to 40 of about 2,517,871 (188)

Phytanic acid, but not pristanic acid, mediates the positive effects of phytol derivatives on brown adipocyte differentiation [PDF]

, 2002
The phytol derivatives phytanic acid and pristanic acid may activate nuclear hormone receptors and influence gene expression and cell differentiation. Phytanic acid induces brown adipocyte differentiation.
Giralt, Marta, Iglesias, Roser, Roser Iglesias, Villarroya, Francesc, Agatha Schluter, Marta Giralt, Francesc Villarroya, Schluter, Agatha   +7 more
core   +1 more source

Refsum disease: a defect in the alpha-oxidation of phytanic acid in peroxisomes

Journal of Lipid Research, 1993
The oxidation of phytanic acid to pristanic acid was previously demonstrated to be deficient in monolayer cultures of skin fibroblasts (Herndon et al. 1969. J. Clin. Invest. 48: 1017-1032).
I Singh, K Pahan, AK Singh, E Barbosa
doaj   +1 more source

Ketoconazole and other imidazole derivatives are potent inhibitors of peroxisomal phytanic acid α-oxidation [PDF]

, 1995
The imidazole antimycotics like ketoconazole, clotrimazole, bifonazole, miconazole and CO, known as powerful inhibitors of cytochrome P-450, are potent inhibitors of peroxisomal phytanic acid α-oxidation to pristanic acid suggesting the possible ...
Khan, Mushfiquddin, Inderjit Singh, Smith, Brian T., Singh, Inderjit, Kalipada Pahan, Pahan, Kalipada, Mushfiquddin Khan, Brian T. Smith   +7 more
core   +1 more source

Phytyl fatty acid esters in vegetables pose a risk for patients suffering from Refsum's disease. [PDF]

PLoS ONE, 2017
Patients suffering from Refsum's disease show mutations in the enzyme necessary for the degradation of phytanic acid. Accumulation of this tetramethyl-branched fatty acid in inner organs leads to severe neurological and cardiac dysfunctions which can ...
Stephanie Krauß, Lea Michaelis, Walter Vetter   +2 more
doaj   +1 more source

Phytanic acid activation in rat liver peroxisomes is catalyzed by long-chain acyl-CoA synthetase

Journal of Lipid Research, 1996
In Refsum disease, disorders of peroxisome biogenesis, and rhizomelic chondrodysplasia punctata, pathological accumulation of phytanic acid results from impaired alpha-oxidation of this branched-chain fatty acid.
P A Watkins, A E Howard, S J Gould, J Avigan, S J Mihalik   +4 more
doaj   +1 more source

Identification of the cytochrome P450 enzymes responsible for the ω-hydroxylation of phytanic acid [PDF]

, 2006
Patients suffering from Refsum disease have a defect in the α-oxidation pathway which results in the accumulation of phytanic acid in plasma and tissues.
Komen, J. C., R.J.A. Wanders, J.C. Komen, Wanders, R.J.A., Wanders, R. J. A., Komen, J.C.   +5 more
core   +1 more source

Pristanic acid and phytanic acid in plasma from patients with peroxisomal disorders: stable isotope dilution analysis with electron capture negative ion mass fragmentography.

Journal of Lipid Research, 1992
A sensitive and selective stable isotope dilution method was developed for the accurate quantitation of pristanic acid and phytanic acid using electron capture negative ion mass fragmentography on pentafluorobenzyl derivatives.
HJ ten Brink, F Stellaard, CM van den Heuvel, RM Kok, DS Schor, RJ Wanders, C Jakobs   +6 more
doaj   +1 more source

Sexually dimorphic metabolism of branched-chain lipids in C57BL/6J mice

Journal of Lipid Research, 2004
Despite the importance of branched chain lipid oxidation in detoxification, almost nothing is known regarding factors regulating peroxisomal uptake, targeting, and metabolism.
Barbara P. Atshaves, H. Ross Payne, Avery L. McIntosh, Shane E. Tichy, David Russell, Ann B. Kier, Friedhelm Schroeder   +6 more
doaj   +1 more source

Slc25a17 Gene Trapped Mice: PMP34 Plays a Role in the Peroxisomal Degradation of Phytanic and Pristanic Acid

Frontiers in Cell and Developmental Biology, 2020
Mice lacking PMP34, a peroxisomal membrane transporter encoded by Slc25a17, did not manifest any obvious phenotype on a Swiss Webster genetic background, even with various treatments designed to unmask impaired peroxisomal functioning. Peroxisomal α- and
Paul P. Van Veldhoven, Evelyn de Schryver, Stephen G. Young, An Zwijsen, Marc Fransen, Marc Espeel, Myriam Baes, Elke Van Ael   +7 more
doaj   +1 more source

Infantile Refsum Disease

Pediatric Neurology Briefs, 1988
Two patients with infantile Refsum (phytanic acid storage) disease were treated at 9 mos and 5½ years of age with a low phytanic acid diet and the effects studied over a 2-yr period or longer in the Depts of Chemical Pathology, Neurology and ...
J Gordon Millichap
doaj   +1 more source