Metabolism of phytanic acid and 3-methyl-adipic acid excretion in patients with adult Refsum disease

Metabolism of phytanic acid and 3-methyl-adipic acid excretion in patients with adult Refsum disease

Adult Refsum disease (ARD) is associated with defective α-oxidation of phytanic acid (PA). ω-Oxidation of PA to 3-methyl-adipic acid (3-MAA) occurs although its clinical significance is unclear. In a 40 day study of a new ARD patient, where the plasma half-life of PA was 22.4 days, ω-oxidation accou...

Full description

Bibliographic Details
Main Authors:	Anthony S. Wierzbicki, Phillip D. Mayne, Matthew D. Lloyd, David Burston, Guam Mei, Margaret C. Sidey, Michael D. Feher, F. Brian Gibberd
Format:	Article
Language:	English
Published:	Elsevier 2003-08-01
Series:	Journal of Lipid Research
Subjects:	organic acid α-oxidation ω-oxidation
Online Access:	http://www.sciencedirect.com/science/article/pii/S0022227520337561

Similar Items

Refsum's disease: a peroxisomal disorder affecting phytanic acid alpha-oxidation.
by: Wierzbicki, A, et al.
Published: (2002)

ω-Hydroxylation of phytanic acid in rat liver microsomes
by: J.C. Komen, et al.
Published: (2004-07-01)

Refsum disease: a defect in the alpha-oxidation of phytanic acid in peroxisomes
by: I Singh, et al.
Published: (1993-10-01)

Phytanic acid oxidation: normal activation and transport yet defective alpha-hydroxylation of phytanic acid in peroxisomes from Refsum disease and rhizomelic chondrodysplasia punctata
by: K Pahan, et al.
Published: (1996-05-01)

Fatty acid profile and physicochemical characterization of buriti oil during storage
by: Juliana de Andrade Mesquita, et al.
Published: (2020-10-01)

Phytanic Acid Intake and Lifestyle Modifications on Quality of Life in Individuals with Adult Refsum Disease: A Retrospective Survey Analysis
by: Jeffrey J. Li, et al.
Published: (2023-05-01)

Adipic Acid Route: Oxidation of Cyclohexene vs. Cyclohexane
by: Ana P. C. Ribeiro, et al.
Published: (2020-12-01)

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
by: Sabine Rönicke, et al.
Published: (2009-11-01)

Biosynthesis of aliphatic plastic monomers with amino residues in Yarrowia lipolytica
by: Gyuyeon Park, et al.
Published: (2023-01-01)

Role of phytanoyl-CoA 2-hydroxylase in phytanic acid metabolism.
by: Lloyd, MD, et al.
Published: (2003)

The Refsum disease marker phytanic acid, a branched chain fatty acid, affects Ca2+ homeostasis and mitochondria, and reduces cell viability in rat hippocampal astrocytes
by: Stefan Kahlert, et al.
Published: (2005-02-01)

Relative contributions of α-, β-, and ω-oxidative pathways to in vitro fatty acid oxidation in rat liver
by: Gabor J. Antony, et al.
Published: (1968-03-01)

Metabolism of phytol-U-14C and phytanic acid-U-14C in the rat
by: Charles E. Mize, et al.
Published: (1966-09-01)

Separation of adipic acid from water-acidic waste of caprolactam production
by: A. A. Sokolova, et al.
Published: (2013-12-01)

ω-3 and ω-6 Polyunsaturated Fatty Acids, Obesity and Cancer
by: Stefania D’Angelo, et al.
Published: (2020-09-01)

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
by: Nicol Kruska, et al.
Published: (2011-08-01)

Phytanic acid oxidation: topographical localization of phytanoyl-CoA ligase and transport of phytanic acid into human peroxisomes.
by: K Pahan, et al.
Published: (1995-05-01)

Chemical composition of fatty acids in spelt and flaxseed pasta
by: Filipović Jelena, et al.
Published: (2016-01-01)

Phytol/Phytanic acid and insulin resistance: potential role of phytanic acid proven by docking simulation and modulation of biochemical alterations.
by: Mohamed M Elmazar, et al.
Published: (2013-01-01)

New spaces and Continuity via ˆΩ-closed sets
by: M. Lellis Thivagar, et al.
Published: (2014-09-01)

Utilization of sterol carrier protein-2 by phytanoyl-CoA 2-hydroxylase in the peroxisomal alpha oxidation of phytanic acid.
by: Mukherji, M, et al.
Published: (2002)

Effects of dietary phytol and phytanic acid in animals
by: Daniel Steinberg, et al.
Published: (1966-09-01)

Modulation of essential fatty acid levels in coconut oil with flaxseed oil
by: A.A. Joshi, et al.
Published: (2023-06-01)

Oxidized hyaluronic acid/adipic acid dihydrazide hydrogel as cell microcarriers for tissue regeneration applications
by: França Carla Giometti, et al.
Published: (2022-12-01)

Association of dietary ω-3 and ω-6 fatty acids intake with cognitive performance in older adults: National Health and nutrition examination Survey (NHANES) 2011–2014
by: Xue Dong, et al.
Published: (2020-03-01)

Coupled action of ozone and UV radiation towards obtaining adipic acid from cyclohexane/one
by: Marek Adam A., et al.
Published: (2024-03-01)

The Effects of Different Fat Sources on Bioproductive Performances and Essential Fatty Acids Composition in Broiler Breast
by: Dragos Sorin Fota, et al.
Published: (2023-10-01)

Biosynthesis of uniformly carbon isotope-labeled docosahexaenoic acid in Crypthecodinium cohnii
by: Pingping Song, et al.
Published: (2020-03-01)

Slc25a17 Gene Trapped Mice: PMP34 Plays a Role in the Peroxisomal Degradation of Phytanic and Pristanic Acid
by: Paul P. Van Veldhoven, et al.
Published: (2020-03-01)

Ratio of omega-6/omega-3 fatty acids of spelt and flaxseed pasta and consumer acceptability
by: Jelena Filipović, et al.
Published: (2016-12-01)

Lower ω-6/ω-3 Polyunsaturated Fatty Acid Ratios Decrease Fat Deposition by Inhibiting Fat Synthesis in Gosling
by: Lihuai Yu, et al.
Published: (2016-10-01)

Phytanic acid, an inconclusive phytol metabolite: A review
by: Muhammad Torequl Islam, et al.
Published: (2023-01-01)

On Fundamental Theorems of Fuzzy Isomorphism of Fuzzy Subrings over a Certain Algebraic Product
by: Alaa Altassan, et al.
Published: (2021-06-01)

Diets with Higher ω-6/ω-3 Ratios Show Differences in Ceramides and Fatty Acid Levels Accompanied by Increased Amyloid-Beta in the Brains of Male APP/PS1 Transgenic Mice
by: Lara Ordóñez-Gutiérrez, et al.
Published: (2021-10-01)

ω-3 and ω-6 Polyunsaturated Fatty Acids Regulate the Proliferation, Invasion and Angiogenesis of Gastric Cancer Through COX/PGE Signaling Pathway
by: Jiachi Ma, et al.
Published: (2022-02-01)

Involvement of cytochrome P450 2E1 in the (ω–1)-hydroxylation of oleic acid in human and rat liver microsomes
by: Fadi Adas, et al.
Published: (1998-06-01)

Response to important considerations when assessing the effect of essential fatty acids on cognitive performance
by: Xue Dong, et al.
Published: (2020-09-01)

ω-Carboxyl variants of 7-ketocholesteryl esters are ligands for β2-glycoprotein I and mediate antibody-dependent uptake of oxidized LDL by macrophages
by: Qingping Liu, et al.
Published: (2002-09-01)

Effects of Low ω6:ω3 Ratio in Sow Diet and Seaweed Supplement in Piglet Diet on Performance, Colostrum and Milk Fatty Acid Profiles, and Oxidative Status
by: Thi Xuan Nguyen, et al.
Published: (2020-11-01)

Selective Oxidation of Cyclohexanone to Adipic Acid Using Molecular Oxygen in the Presence of Alkyl Nitrites and Transition Metals as Catalysts
by: Dawid Lisicki, et al.
Published: (2023-08-01)