Current views of fatty acid oxidation and ketogenesis : from organelles to point mutations

Biogenesis of the Rat Liver Mitochondrial Carnitine Palmitoyltransferase I....Pages 1-16
Subcellular Distributuon of Mitochondrial Carnitine Palmitoyltransferase I in Rat Liver....Pages 17-25
Topology of Hepatic Mitochondrial Carnitine Palmitoyltransferase I....Pages 27-42
Possible Involvement of Cytoskeletal Components in the Control of Hepatic Carnitine Palmitoyltransferase I Activity....Pages 43-52
Effects of 3-Thia Fatty Acids on β-Oxidation and Carnitine Palmitoylatransferase I Activity in Cultured Rat Hepatocytes....Pages 53-57
Carnitine Acyltransferases and Associated Transport Processes in the Endoplasmic Reticulum....Pages 59-67
Reciprocal Enzymatic Interference of Carnitine Palmitoyltransferase I and Glycerol-3-Phosphate Acyltransferase in Purified Liver Mitochondria....Pages 69-78
Characterization of a Response Element for Peroxisomal Proliferator Activated Receptor (PPRE) in Human Muscle-type Carnitine Palmitoyltransferase I....Pages 79-85
Kinetic Investigation of Carnitine Palmitoyltransferases in Homogenates of Human Skeletal Muscle Using L-amino-Carnitine and Malonyl-CoA....Pages 87-93
Processing of Carnitine Octanoyl Transferase pre-mRNAs by cis and trans -splicing....Pages 95-102
Selective Modulation of Carnitine Long-chain Acyltransferase Activities....Pages 103-109
Confocal Laser Scanning Microscopy of Human Skin Fibroblasts Showing Transient Expression of a Green Fluorescent Carnitine Palmitoyltransferase 1 Fusion Protein....Pages 111-115
Carnitine Biosynthesis....Pages 117-124
Hypolipidemic 3-Thia Fatty Acids....Pages 125-132
Molecular Mechanisms of Fatty Acid β -Oxidation Enzyme Catalysis....Pages 133-143
Control of Mitochondrial β -Oxidation at the Levels of [NAD + ]/[NADH] and CoA Acylation....Pages 145-154
Production and Export of Acylcarnitine Esters by Neonatal Rat Hepatocytes....Pages 155-159
Tissue Specific Differences in Intramitochondrial Control of β -Oxidation....Pages 161-168
Endotoxin-Induced Changes in Very-Low-Density Lipoprotein and Myocardial Utilisation of Triacylglycerol from Abnormal VLDL in the Rat....Pages 169-175
Effect of Valproic Acid on the Expression of Acyl-CoA Dehydrogenases in Various Tissues....Pages 177-189
Formation of a Human “Electron Transferring Flavoprotein”....Pages 191-194
Cloning and Regulation of Peroxisome Proliferator-Induced Acyl-CoA Thioesterases From Mouse Liver....Pages 195-200
Metabolic Effects of 3-Thia Fatty Acid in Cancer Cells....Pages 201-204
Poorly Oxidizable Fatty Acid Analogues Inhibit the Proliferation of Cancer Cells in Culture....Pages 205-210
The Role of PPARα as a“lipostat” Transcription Factor....Pages 211-220
The Hypolipidaemic Effect of EPA is Potentiated by 2- and 3-Methylation....Pages 221-226
Is it Time to Reconsider the Role of CPT I in Control of Hepatic Ketogenesis?....Pages 227-232
Developmental Comparison of Human and Rat Hepatic Mitochondrial 3-Hydroxy-3-Methylglutaryl-CoA Synthase....Pages 233-239
Regulation of the Ketogenic Enzyme Mitochondrial 3-Hydroxy-3-Methylglutaryl-COA Synthase in Astrocytes and Meningeal Fibroblasts....Pages 241-251
Studies on Regulation of the Peroxisomal β -Oxidation at the 3-Ketothiolase Step....Pages 253-259
Role and Organization of Peroxisomal β -Oxidation....Pages 261-272
Hepatic α -Oxidation of Phytanic Acid....Pages 273-281
Functions and Dysfunctions of Peroxisomes in Fatty Acid α - and β -Oxidation....Pages 283-299
Enzymology of β -Oxidation of (Poly)unsaturated Fatty Acids....Pages 301-309
The Effect of β-Oxidable and Non- β-Oxidable Thia Fatty Acids on Fatty Acid Metabolism....Pages 311-314
EPA and DHA Possess Different Metabolic Properties....Pages 315-320
The Use of [9,10- 3 H]Myristate, [9,10- 3 H]Palmitate and [9,10- 3 H]Oleate for the Detection and Diagnosis of Medium and Long-Chain Fatty Acid Oxidation Disorders in Intact Cultured Fibroblasts....Pages 321-325
Rapid Diagnosis of Organic Acidemias and Fatty-acid Oxidation Defects by Quantitative Electrospray Tandem-MS Acyl-Carnitine Analysis in Plasma....Pages 327-337
Genetics of Carnitine Palmitoyltransferase II Deficiencies....Pages 339-345
Identification of a Missense Mutation in a Patient with Lethal Carnitine Acyl-Carnitine Carrier Deficiency....Pages 347-351
MCAD Deficiency....Pages 353-363
D-Hydroxyacyl-CoA Dehydrogenase Deficiency....Pages 365-369
Phytanoyl-CoA Hydroxylase Deficiency....Pages 371-376
Rationale for a Conditional Knockout Mouse Model to Study Carnitine Palmitoyltransferase I Deficiencies....Pages 377-385
Biochemical Characterisation of Mutations of Human Medium-Chain Acyl-CoA Dehydrogenase....Pages 387-393
Lessons Learned from The Mouse Model of Short-Chain Acyl-CoA Dehydrogenase Deficiency....Pages 395-402