Results 131 to 140 of about 2,517,871 (188)

α-Methylacyl-CoA Racemase Deficiency in a Patient with Ataxia, Spasticity, and Segmental Dystonia. [PDF]

open access: yesMov Disord Clin Pract
Rashedi R   +5 more
europepmc   +1 more source

Phytanic acid metabolism in health and disease

open access: yesBiochimica Et Biophysica Acta - Molecular and Cell Biology of Lipids, 2011
Phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) is a branched-chain fatty acid which cannot be beta-oxidized due to the presence of the first methyl group at the 3-position. Instead, phytanic acid undergoes alpha-oxidation to produce pristanic acid (2,6,10,14-tetramethylpentadecanoic acid) plus CO(2).
Ronald Wanders, Sacha Ferdinandusse
exaly   +6 more sources

Phytanic acid—an overlooked bioactive fatty acid in dairy fat?

open access: yesAnnals of the New York Academy of Sciences, 2010
Phytanic acid is a multibranched fatty acid with reported retinoid X receptor (RXR) and peroxisome proliferator‐activated receptor‐α (PPAR‐α) agonist activity, which have been suggested to have preventive effects on metabolic dysfunctions. Serum level in man is strongly correlated to the intake of red meat and dairy products and the concentration in ...
Lars I Hellgren
exaly   +4 more sources

The metabolism of phytanic acid and pristanic acid in man: A review

open access: yesJournal of Inherited Metabolic Disease, 1998
AbstractThe branched‐chain fatty acid phytanic acid is a constituent of the diet, present in diary products, meat and fish. Degradation of this fatty acid in the human body is preceded by activation to phytanoyl‐CoA and starts withone cycle of α‐oxidation. Intermediates in this pathway are 2‐hydroxy‐phytanoyl‐CoA and pristanal; the product is pristanic
Verhoeven, N.M.   +4 more
openaire   +4 more sources

Phytanic Acid Metabolism in Health and Disease

open access: yes, 2020
Phytanic 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
Wanders, Ronald J. A.   +1 more
openaire   +2 more sources

Phytanic acid impairs mitochondrial respiration through protonophoric action [PDF]

open access: yesCellular and Molecular Life Sciences, 2007
Contains fulltext : 51928.pdf (Publisher’s version ) (Open Access)Refsum disease is a rare, inherited neurodegenerative disorder characterized by accumulation of the dietary branched-chain fatty acid phytanic acid in plasma and tissues ...
Ronald Wanders, Werner J H Koopman
exaly   +2 more sources

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