Metabolism of branched chain fatty acids in peroxisomal disorders (original) (raw)

Measurement of peroxisomal fatty acid β-oxidation in cultured human skin fibroblasts

C. Roermund

Journal of Inherited Metabolic Disease, 1995

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Metabolism of saturated and polyunsaturated very-long-chain fatty acids in fibroblasts from patients with defects in peroxisomal β-oxidation

Jackie Street

Biochemical Journal, 1990

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Peroxisomal fatty acid beta-oxidation in relation to the accumulation of very long chain fatty acids in cultured skin fibroblasts from patients with Zellweger syndrome and other peroxisomal disorders

Carlo van Roermund

Journal of Clinical Investigation, 1987

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Metabolism of saturated and polyunsaturated fatty acids by normal and Zellweger syndrome skin fibroblasts

Jackie Street

Biochemical Journal, 1989

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The β-oxidation of arachidonic acid and the synthesis of docosahexaenoic acid are selectively and consistently altered in skin fibroblasts from three Zellweger patients versus X-adrenoleukodystrophy, Alzheimer and control subjects

Nadia Papini

Neuroscience Letters, 1998

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Medium-chain fatty acids undergo elongation before �-oxidation in fibroblasts

Yasmeen Butt

Biochem Biophys Res Commun, 2006

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Effect of Ascorbic and Dehydroascorbic Acids on Lipid Composition of C 3 H/10T1/2 Cells

Victoria L. Ibric

Annals of the New York Academy of Sciences, 1987

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Alkylthioacetic acid (3-thia fatty acids) — a new group of non-β-oxidizable, peroxisome-inducing fatty acid analogues. I. A study on the structural requirements for proliferation of peroxisomes and mitochondria in rat liver

R. Berge

Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1989

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Medium-chain fatty acids undergo elongation before β-oxidation in fibroblasts

Yasmeen Butt

Biochemical and Biophysical Research Communications, 2006

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Alkylthioacetic acid (3-thia fatty acids)--a new group of non-beta-oxidizable, peroxisome-inducing fatty acid analogues. I. A study on the structural requirements for proliferation of peroxisomes and mitochondria in rat liver

Rolf Berge

Biochimica et biophysica acta, 1989

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Morphologic effects of sulfur-substituted fatty acids on rat hepatocytes with special reference to proliferation of peroxisomes and mitochondria

Rolf Berge

Journal of Structural Biology, 1990

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Very long chain fatty acids activate NADPH oxidase in human dermal fibroblasts

Milad Bitar

Cell Biochemistry and Function, 2004

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Arachidonic and docosahexaenoic acids are biosynthesized from their 18-carbon precursors in human infants

Paul Mena

Proceedings of the National Academy of Sciences, 1996

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Conversion of hexadecanoic acid to hexadecenoic acid by rat Delta6-desaturase

Hervé Guillou, Sophie Jan

The Journal of Lipid Research, 2003

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Inhibitory effect of 3-hydroxyacyl-CoAs and other long-chain fatty acidβ-oxidation intermediates on mitochondrial oxidative phosphorylation

Jos Ruiter

Journal of Inherited Metabolic Disease, 1996

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Non enzymatic metabolites of polyunsaturated fatty acids: friend or foe

Valérie Bultel-Poncé

OCL, 2015

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Essential fatty acids: biochemistry, physiology and pathology

Undurti Das

Biotechnology Journal, 2006

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Five decades with polyunsaturated Fatty acids: chemical synthesis, enzymatic formation, lipid peroxidation and its biological effects

Angel Catalá

Journal of lipids, 2013

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Effect of polyunsaturated fatty acids and antioxidants on lipid peroxidation in tissue cultures

Victor Gavino

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Phytanic acid and pristanic acid are oxidized by sequential peroxisomal and mitochondrial reactions in cultured fibroblasts

Diane Roe

Journal of Lipid Research, 1998

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Inhibitory effect of 3-hydroxyacyl-CoAs and other long-chain fatty acid )J-oxidation intermediates on mitochondrial oxidative phosphorylation

Jos Ruiter

J Inherit Metab Dis, 1996

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Alkylthio acetic acids (3-thia fatty acids)—A new group of non-β-oxidizable peroxisome-inducing fatty acid analogues—II. Dose-response studies on hepatic peroxisomal- and mitochondrial changes and long-chain fatty acid metabolizing enzymes in rats

Rolf Berge

Biochemical Pharmacology, 1989

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Up-regulated Δ9-desaturase gene expression by hypolipidemic peroxisome-proliferating fatty acids results in increased oleic acid content in liver and VLDL: Accumulation of a Δ9-desaturated metabolite of tetradecylthioacetic acid

Rolf Berge

The Journal of Lipid Research

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Effects of odd-numbered medium-chain fatty acids on the accumulation of long-chain 3-hydroxy-fatty acids in long-chain L-3-hydroxyacyl CoA dehydrogenase and mitochondrial trifunctional protein deficient skin fibroblasts

Yasmeen Butt

Molecular Genetics and Metabolism, 2004

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Peroxisomal Fatty Acid β-Oxidation in Relation to Adrenoleukodystrophy

C. Roermund

Developmental Neuroscience, 1991

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Selective modification of rat hepatic microsomal fatty acid chain elongation and desaturation by fibrates: relationship with peroxisome proliferation

Manuel Merlos

British Journal of Pharmacology, 1995

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Comparison of fatty acid α-oxidation by rat hepatocytes and by liver microsomes fortified with NADPH, Fe3+ and phosphate

Guy Mannaerts, P Van Veldhoven

Lipids, 1994

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Defective oxidation of pristanic acid by fibroblasts from patients with disorders in propionic acid metabolism

harinder singh

Clinical Genetics, 2008

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alpha-Oxidation of 3-Methyl-Substituted Fatty Acids in Rat Liver. Production of Formic Acid Instead of CO2, Cofactor Requirements, Subcellular Localization and Formation of a 2-Hydroxy-3-Methylacyl-Coa Intermediate

Guy Mannaerts, P Van Veldhoven

European Journal of Biochemistry, 1996

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Manipulation of the fate of long chain polyunsaturated fatty acids in cultured cells

Patrizia Risè

Prostaglandins, Leukotrienes and Essential Fatty Acids, 1997

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