Results 171 to 180 of about 4,018 (220)
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Phytanic acid α‐hydroxylation by bacterial cytochrome P450
Lipids, 1998AbstractFatty acid α‐hydroxylase, a cytochrome P450 enzyme, from Sphingomonas paucimobilis, utilizes various straight‐chain fatty acids as substrates. We investigated whether a recombinant fatty acid α‐hydroxylase is able to metabolize phytanic acid, a methyl‐branched fatty acid.
I, Matsunaga +3 more
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Neurology, 1983
Heredopathia atactica polyneuritiformis is a biochemically defined disease with a specific dietary treatment. It is an autosomal inborn error of metabolism. The phytanic acid is of exogenous origin and stems mainly from preformed phytanic acid in foods.
G, Djupesland, G, Flottorp, S, Refsum
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Heredopathia atactica polyneuritiformis is a biochemically defined disease with a specific dietary treatment. It is an autosomal inborn error of metabolism. The phytanic acid is of exogenous origin and stems mainly from preformed phytanic acid in foods.
G, Djupesland, G, Flottorp, S, Refsum
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Hepatic α-Oxidation of Phytanic Acid
2002Synthetic 3-methyl-branched chain fatty acids were used to decipher the breakdown of phytanic acid. Based on results obtained in intact or permeabilized rat hepatocytes, rat liver homogenates or subcellular fractions, a revised alpha-oxidation pathway is proposed which appears to be functioning in man as well.
P P, Van Veldhoven +3 more
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Phytanic acid α-oxidation in rat liver mitochondria
Biochimica et Biophysica Acta (BBA) - General Subjects, 1994The alpha-oxidation of phytanic acid in rat liver is a mitochondrial function. The inhibition of phytanic acid oxidation activity by inhibitors of acyl-CoA ligases (Naproxen and Triacsin C) and that of carnitine acyltransferase I (2-(5-(4-chlorophenyl)pentyl)oxirane-2 carboxylic acid (POCA) and 2-bromopalmitate) and increase in phytanic acid oxidation ...
K, Pahan, S, Gulati, I, Singh
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Occurrence of Phytanic Acid in Rumen Bacteria
Nature, 1966THE branched-chain fatty acid 3,7,11,15-tetramethyl-hexadecanoic acid (phytanic acid) has been isolated and identified in butterfat1–4, sheep fat5, ox fat6, ox serum7,8, crude petroleum9 and also in the tissues, serum and urine of humans afflicted with the rare disease referred to as Refsum's syndrome10–12 (heredopathia atactica polyneuritiformis ...
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Phytanic and Pristanic Acid Are Naturally Occuring Ligands
2003Phytanic acid (3,7,11,15 tetramethylhexadecanoic acid) is an isoprenoid-derived 3-methyl fatty acid, which is solely derived from external sources. This branched-chain fatty acid originates from the phytol side chain of chlorophyll. Microorganisms, which are present in the rumen of ruminants, are thought to release phytol from chlorophyll, after which ...
Zomer, Anna W. M. +2 more
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Phytanic Acid Metabolism in Health and Disease
2020Phytanic acid is one of the most common branched-chain fatty acids in the human diet. It cannot be synthesized endogenously, at least in humans, and it is especially high in fat-containing foods of animal origin; it also occurs in animals higher up the food chain, such as mollusks, fish, and whales. Degradation of phytanic acid occurs via a specialized
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Ophthalmic Manifestations of Infantile Phytanic Acid Storage Disease
Archives of Ophthalmology, 1984Two patients had infantile phytanic acid storage disease. Patient 1 had nystagmus from early infancy, epicanthal folds, esotropia, and a pigmentary retinopathy. The second case had similar manifestations; however, no nystagmus was present. Both patients were hypotonic as infants, had a severe hearing impairment, and were moderately severely ...
R G, Weleber +4 more
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Phytanic acid in tissue lipids in Refsum's disease
Clinica Chimica Acta, 1969Abstract Phytanic acid levels in a variety of tissues and in cerebrospinal fluid in Refsum's disease have been determined. Fatty acid profiles of cholesterol ester, lecithin and triglycerides in the liver, kidney and heart were estimated. Considerable accumulation of phytanic acid in the heart was found, together with raised levels of cholesterol in ...
T R, Skrbic, J N, Cumings
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Very-Long-Chain Fatty Acids and Phytanic Acid
2008The peroxisomes are subcellular organelles with a variety of biochemical functions, amongst which a system for beta-oxidation of very long-chain (C22-C26) fatty acids (VLCFA) and a system for alpha-oxidation of branched-chain fatty acids such as phytanic acid.
Wanders, R. J. A., Duran, M.
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