Results 41 to 50 of about 4,018 (220)

Phytanic acid and pristanic acid are oxidized by sequential peroxisomal and mitochondrial reactions in cultured fibroblasts

Journal of Lipid Research, 1998
The relationship between peroxisomal and mitochondrial oxidation of the methyl branched fatty acids, phytanic acid and pristanic acid, was studied in normal and mutant human skin fibroblasts with established enzyme deficiencies.
Nanda M. Verhoeven, Diane S. Roe, Robert M. Kok, Ronald J.A. Wanders, Cornelis Jakobs, Charles R. Roe   +5 more
doaj   +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

ACBD5 deficiency causes a defect in peroxisomal very long-chain fatty acid metabolism [PDF]

, 2016
Background Acyl-CoA binding domain containing protein 5 (ACBD5) is a peroxisomal membrane protein with a cytosolic acyl-CoA binding domain. Because of its acyl-CoA binding domain, ACBD5 has been assumed to function as an intracellular carrier of acyl ...
Falkenberg, K, KF, Ferdinandusse, S, SF, Jones, R, RJ, Koster, J, JK, Mooyer, PM, Pizzino, A, AP, Schrader, M, van Roermund, C, C, Vanderver, A, AV, Wanders, R, RW, Waterham, H, HW   +10 more
core   +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

Metabolome response to temperature-induced virulence gene expression in two genotypes of pathogenic Vibrio parahaemolyticus [PDF]

, 2016
Relative concentration of metabolites identified in Vibrio parahaemolyticus ATCC17802. (XLSX 113 kb)
Bo Feng, Weijia Zhang, Yingjie Pan, Yong Zhao, Zhuoran Guo   +4 more
core   +3 more sources

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

Different types of peroxisomes in human duodenal epithelium [PDF]

, 1991
Peroxisomes are ubiquitous organelles containing enzyme sequences for beta-oxidation of fatty acids, synthesis of bile acids, and ether phospholipids.
Roels, Frank, Espeel, Marc, Pauwels, M, De Craemer, Dirk, Egberts, H, Van der Spek, P   +5 more
core   +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