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

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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A new, simple assay for long-chain acyl-CoA dehydrogenase in cultured skin fibroblasts using stable isotopes and GC-MS

Hugo Heymans

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, 1992

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Metabolic and Phenotypic Characterization of Human Skin Fibroblasts After Forcing Oxidative Capacity

Cláudia Deus

Toxicological sciences : an official journal of the Society of Toxicology, 2018

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Evaluation of metabolic pathway activity in cultured skin fibroblasts and blood leukocytes

Rima Rozen

Clinica Chimica Acta, 1977

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Acyl-CoA: dihydroxyacetone phosphate acyltransferase in human skin fibroblasts: study of its properties using a new assay method

rob ofman

Biochimica Et Biophysica Acta (bba) - Lipids and Lipid Metabolism, 1986

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The Oxidation of Fatty Acids by Rabbit Reticulocytes and Their Isolated Mitochondria

Frank Gellerich

European Journal of Biochemistry, 1972

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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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Mitochondrial and metabolic remodeling in human skin fibroblasts in response to glucose availability

Cláudia Deus

2021

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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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Accumulation of 3-Hydroxy-Fatty Acids in the Culture Medium of Long-Chain l-3-Hydroxyacyl CoA Dehydrogenase (LCHAD) and Mitochondrial Trifunctional Protein-Deficient Skin Fibroblasts: Implications for Medium Chain Triglyceride Dietary Treatment of LCHAD Deficiency

Yasmeen Butt

Pediatric Research, 2003

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

Milad Bitar

Cell Biochemistry and Function, 2004

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Regulation of the mitochondrial ATP-synthase in human fibroblasts

Anibh Das

Clinica Chimica Acta, 1994

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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 fatty acids by normal and Zellweger syndrome skin fibroblasts

Jackie Street

Biochemical Journal, 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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Analysis of fatty acid oxidation intermediates in cultured fibroblasts to detect mitochondrial oxidation disorders

morteza pourfarzam

Clinical Chemistry

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Polyunsaturated fatty acid accumulation in the lipids of cultured fibroblasts and smooth muscle cells

Victor Gavino

Journal of lipid …, 1981

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Measurement of short-chain acyl-CoA dehydrogenase (SCAD) in cultured skin fibroblasts with hexanoyl-CoA as a competitive inhibitor to eliminate the contribution of medium-chain acyl-CoA dehydrogenase

Hugo Heymans

Clinica Chimica Acta, 1994

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Inhibition of oxidative phosphorylation by palmitoyl-CoA in digitonin permeabilized fibroblasts: implications for long-chain fatty acid β-oxidation disorders

Jos Ruiter

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, 1995

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Identification of fatty acid oxidation disorder patients with lowered acyl-CoA thioesterase activity in human skin fibroblasts

rob ofman

European Journal of Clinical Investigation, 2005

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Raising plasma fatty acid concentration induces increased biogenesis of mitochondria in skeletal muscle

Pablo M Garcia-Roves

Proceedings of the National Academy of Sciences, 2007

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Effect of injected glucagon or fatty acids on mitochondrial ATPase

Belen Garrido

Archives of Biochemistry and Biophysics, 1984

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Correction of Fatty Acid Oxidation in Carnitine Palmitoyl Transferase 2–Deficient Cultured Skin Fibroblasts by Bezafibrate

Jean Bastin

Pediatric Research, 2003

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Analysis of organic acids after incubation with (16- 2 H 3 )palmitic acid in fibroblasts from patients with mitochondrial β-oxidation defects

Jose Henry Osorio

Journal of Inherited Metabolic Disease, 2000

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Metabolic fate of extracellular NAD in human skin fibroblasts

Giuseppe Pompucci

Journal of Cellular Biochemistry, 2001

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Impaired Mitochondrial Fatty Acid Oxidative Flux in Fibroblasts from a Patient with Malonyl-CoA Decarboxylase Deficiency

Michael Bennett

Molecular Genetics and Metabolism, 2001

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Peroxisomal Fatty Acid β-Oxidation in Relation to Adrenoleukodystrophy

C. Roermund

Developmental Neuroscience, 1991

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Fatty acid oxidation in peripheral blood cells: characterization and use for the diagnosis of defects of fatty acid oxidation

morteza pourfarzam

Pediatric …, 1995

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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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Genetic Deficiency of Medium-Chain Acyl Coenzyme A Dehydrogenase: Studies in Cultured Skin Fibroblasts and Peripheral Mononuclear Leukocytes

Barbara Corkey

Pediatric Research, 1985

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Metabolism of branched chain fatty acids in peroxisomal disorders

harinder singh

Journal of Inherited Metabolic Disease, 1990

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