Results 21 to 30 of about 2,517,871 (188)
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
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Serum phytanic and pristanic acid levels and prostate cancer risk in Finnish smokers [PDF]
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 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
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Phytanic acid is ligand and transcriptional activator of murine liver fatty acid binding protein
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
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
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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
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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
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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
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Metabolism of phytol-U-14C and phytanic acid-U-14C in the rat
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
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Effectiveness and Safety of Personalized Cholic Acid Treatment in Patients With Bile Acid Synthesis Defects. [PDF]
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

