Results 21 to 30 of about 2,517,871 (188)

Phytanic acid oxidation: normal activation and transport yet defective alpha-hydroxylation of phytanic acid in peroxisomes from Refsum disease and rhizomelic chondrodysplasia punctata

open access: yesJournal of Lipid Research, 1996
In humans the oxidation of phytanic acid is a peroxisomal function. To understand the possible mechanisms for the pathognomic accumulation of phytanic acid in plasma and body fluids of Refsum disease (RD) and rhizomelic chondrodysplasia punctata (RCDP ...
K Pahan, M Khan, I Singh
doaj   +1 more source

Serum phytanic and pristanic acid levels and prostate cancer risk in Finnish smokers [PDF]

open access: yes, 2014
Phytanic acid is a saturated branched-chain fatty acid found predominantly in red meat and dairy products, and may contribute to the elevated risks of prostate cancer associated with higher consumption of these foods.
D. Albanes (7650236)   +6 more
core   +7 more sources

The Refsum disease marker phytanic acid, a branched chain fatty acid, affects Ca2+ homeostasis and mitochondria, and reduces cell viability in rat hippocampal astrocytes

open access: yesNeurobiology of Disease, 2005
The saturated branched chain fatty acid, phytanic acid, a degradation product of chlorophyll, accumulates in Refsum disease, an inherited peroxisomal disorder with neurological clinical features. To elucidate the pathogenic mechanism, we investigated the
Stefan Kahlert   +2 more
doaj   +1 more source

Phytanic acid is ligand and transcriptional activator of murine liver fatty acid binding protein

open access: yesJournal of Lipid Research, 1999
Branched-chain phytanic acid is metabolized in liver peroxisomes. Sterol carrier protein 2/sterol carrier protein x (SCP2/SCPx) knockout mice, which develop a phenotype with a deficiency in phytanic acid degradation, accumulate dramatically high ...
Christian Wolfrum   +6 more
doaj   +1 more source

ω-Hydroxylation of phytanic acid in rat liver microsomes

open access: yesJournal of Lipid Research, 2004
The 3-methyl-branched fatty acid phytanic acid is degraded by the peroxisomal α-oxidation route because the 3-methyl group blocks β-oxidation. In adult Refsum disease (ARD), peroxisomal α-oxidation is defective, which is caused by mutations in the gene ...
J.C. Komen, M. Duran, R.J.A. Wanders
doaj   +1 more source

Pristanic acid and phytanic acid: naturally occurring ligands for the nuclear receptor peroxisome proliferator-activated receptor α

open access: yesJournal of Lipid Research, 2000
Phytanic acid and pristanic acid are branched-chain fatty acids, present at micromolar concentrations in the plasma of healthy individuals. Here we show that both phytanic acid and pristanic acid activate the peroxisome proliferator-activated receptor α (
Anna W.M. Zomer   +5 more
doaj   +1 more source

Phytanic acid and pristanic acid, branched-chain fatty acids associated with Refsum disease and other inherited peroxisomal disorders, mediate intracellular Ca2+ signaling through activation of free fatty acid receptor GPR40

open access: yesNeurobiology of Disease, 2011
The accumulation of the two branched-chain fatty acids phytanic acid and pristanic acid is known to play an important role in several diseases with peroxisomal impairment, like Refsum disease, Zellweger syndrome and α-methylacyl-CoA racemase deficiency ...
Nicol Kruska, Georg Reiser
doaj   +1 more source

The influence of the branched-chain fatty acids pristanic acid and Refsum disease-associated phytanic acid on mitochondrial functions and calcium regulation of hippocampal neurons, astrocytes, and oligodendrocytes

open access: yesNeurobiology of Disease, 2009
Pristanic acid and phytanic acid are branched-chain fatty acids, which play an important role in diseases with peroxisomal impairment, like Refsum disease (MIM 266500), Zellwegers syndrome and α-methylacyl-CoA racemase deficiency (MIM 604489).
Sabine Rönicke   +3 more
doaj   +1 more source

Metabolism of phytol-U-14C and phytanic acid-U-14C in the rat

open access: yesJournal of Lipid Research, 1966
The metabolism of uniformly-labeled 14C-phytol, 14C-phytenic acid, and 14C-phytanic acid was studied in the rat. Conversion of both phytol and phytenic acid to phytanic acid was demonstrated.
Charles E. Mize   +4 more
doaj   +1 more source

Effectiveness and Safety of Personalized Cholic Acid Treatment in Patients With Bile Acid Synthesis Defects. [PDF]

open access: yesJ Inherit Metab Dis
ABSTRACT Bile acid synthesis defects (BASDs) comprise a group of rare, often severe, metabolic disorders. Bile acid replacement therapy decreases toxic bile acid intermediates production and improves biochemical profiles, potentially delaying or stabilizing disease progression.
Polak Y   +8 more
europepmc   +2 more sources

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