New insights in peroxisomal beta-oxidation

Thesis

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Titlepage

Contents

Abbreviations

Chapter 1 Introduction

Chapter 2 Peroxisomal β-oxidation: a review

Chapter 3 Molecular cloning and expression of human carnitine octanoyltransferase (COT): evidence for its role in the peroxisomal β-oxidation of branched-chain fatty acids.

Chapter 4 Peroxisomal fatty acid oxidation disorders and 58 kDa sterol carrier protein X (SCPx): activity measurements in liver and fibroblasts using a newly developed method.

Chapter 5 Reinvestigation of peroxisomal 3-ketoacyl-CoA thiolase deficiency: identification of the true defect at the level of D-bifunctional protein.

Chapter 6 Mutations in the gene encoding peroxisomal α-methylacyl-CoA racemase cause adult-onset sensory motor neuropathy.

Chapter 7 Subcellular localization and physiological role of α-methylacyl-CoA racemase.

Chapter 8 Plasma analysis of di- and trihydroxycholestanoic acid diastereomers in peroxisomal α-methylacyl-CoA racemase deficiency

Chapter 9 Stereochemistry of the peroxisomal branched-chain fatty acid α- and β-oxidation systems in patients suffering from different peroxisomal disorders.

Chapter 10 Identification of the peroxisomal β-oxidation enzymes involved in the biosynthesis of docosahexaenoic acid.

Summary & Samenvatting

Dankwoord

List of publications

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