Current Views of Fatty Acid Oxidation and Ketogenesis

In September 1991, Victor Zammit and I were in the Department of Biochemistry, the University of Cambridge, discussing ou r collaborative research project when we real ized thepotential value and need fora conference specifically concerned with fatty acid oxidation and ketogenesis. Thei dea, onceseeded, was indulged and flourished into the first Fatty Acid Oxidation & Ketogenesis (FAOx&K) Conference t hatwas held in the Department at Eastertime, 1992. Itw as attended byc olleagues mainly from the U. K. , France and Spain. From these modest beginnings atradition for holding a conference every second yearh asgrown and this Book results from the 4th International FAOx&K Conference that was held in London at the Institute of Child Health & Great Ormond Street Hospital forChildren NHSTrust, University College London Medical School in the new Conference Suite and was attended by colleagues from over twenty c ountries and five different c ontinents. I would like to thank allm y colleagues who havec ontributed to the conferences and, mos t importantly, to this Book. The first two c onferences were held in the University of Cambridge and were orga nized entirely by me but Simon Eaton, who came to work in London with me in Febru ary 1997,b ecame Conference Secretary andco organized the last two meetings. His contribution tot he conferences has been invaluable and w ithout hisdedicated help and effort neither the latermeetings n ort his Book would haveb een possible.

Zusammenfassung
Bringing together biochemical, genetic, molecular biology, and clinical approaches to the study of fatty acid oxidation, this text includes late-1990s research from most of the major groups working in this field.

Inhalt
Biogenesis of the Rat Liver Mitochondrial Carnitine Palmitoyltransferase I.- Subcellular Distributuon of Mitochondrial Carnitine Palmitoyltransferase I in Rat Liver.- Topology of Hepatic Mitochondrial Carnitine Palmitoyltransferase I.- Possible Involvement of Cytoskeletal Components in the Control of Hepatic Carnitine Palmitoyltransferase I Activity.- Effects of 3-Thia Fatty Acids on ?-Oxidation and Carnitine Palmitoylatransferase I Activity in Cultured Rat Hepatocytes.- Carnitine Acyltransferases and Associated Transport Processes in the Endoplasmic Reticulum.- Reciprocal Enzymatic Interference of Carnitine Palmitoyltransferase I and Glycerol-3-Phosphate Acyltransferase in Purified Liver Mitochondria.- Characterization of a Response Element for Peroxisomal Proliferator Activated Receptor (PPRE) in Human Muscle-type Carnitine Palmitoyltransferase I.- Kinetic Investigation of Carnitine Palmitoyltransferases in Homogenates of Human Skeletal Muscle Using L-amino-Carnitine and Malonyl-CoA.- Processing of Carnitine Octanoyl Transferase pre-mRNAs by cis and trans-splicing.- Selective Modulation of Carnitine Long-chain Acyltransferase Activities.- Confocal Laser Scanning Microscopy of Human Skin Fibroblasts Showing Transient Expression of a Green Fluorescent Carnitine Palmitoyltransferase 1 Fusion Protein.- Carnitine Biosynthesis.- Hypolipidemic 3-Thia Fatty Acids.- Molecular Mechanisms of Fatty Acid ?-Oxidation Enzyme Catalysis.- Control of Mitochondrial ?-Oxidation at the Levels of [NAD+]/[NADH] and CoA Acylation.- Production and Export of Acylcarnitine Esters by Neonatal Rat Hepatocytes.- Tissue Specific Differences in Intramitochondrial Control of ?-Oxidation.- Endotoxin-Induced Changes in Very-Low-Density Lipoprotein and Myocardial Utilisation of Triacylglycerol fromAbnormal VLDL in the Rat.- Effect of Valproic Acid on the Expression of Acyl-CoA Dehydrogenases in Various Tissues.- Formation of a Human Electron Transferring Flavoprotein.- Cloning and Regulation of Peroxisome Proliferator-Induced Acyl-CoA Thioesterases From Mouse Liver.- Metabolic Effects of 3-Thia Fatty Acid in Cancer Cells.- Poorly Oxidizable Fatty Acid Analogues Inhibit the Proliferation of Cancer Cells in Culture.- The Role of PPAR? as alipostat Transcription Factor.- The Hypolipidaemic Effect of EPA is Potentiated by 2- and 3-Methylation.- Is it Time to Reconsider the Role of CPT I in Control of Hepatic Ketogenesis?.- Developmental Comparison of Human and Rat Hepatic Mitochondrial 3-Hydroxy-3-Methylglutaryl-CoA Synthase.- Regulation of the Ketogenic Enzyme Mitochondrial 3-Hydroxy-3-Methylglutaryl-COA Synthase in Astrocytes and Meningeal Fibroblasts.- Studies on Regulation of the Peroxisomal ?-Oxidation at the 3-Ketothiolase Step.- Role and Organization of Peroxisomal ?-Oxidation.- Hepatic ?-Oxidation of Phytanic Acid.- Functions and Dysfunctions of Peroxisomes in Fatty Acid ?- and ?-Oxidation.- Enzymology of ?-Oxidation of (Poly)unsaturated Fatty Acids.- The Effect of ?-Oxidable and Non-?-Oxidable Thia Fatty Acids on Fatty Acid Metabolism.- EPA and DHA Possess Different Metabolic Properties.- The Use of [9,10-3H]Myristate, [9,10-3H]Palmitate and [9,10-3H]Oleate for the Detection and Diagnosis of Medium and Long-Chain Fatty Acid Oxidation Disorders in Intact Cultured Fibroblasts.- Rapid Diagnosis of Organic Acidemias and Fatty-acid Oxidation Defects by Quantitative Electrospray Tandem-MS Acyl-Carnitine Analysis in Plasma.- Genetics of Carnitine Palmitoyltransferase II Deficiencies.- Identification of a Missense Mutation in a Patient withLethal Carnitine Acyl-Carnitine Carrier Deficiency.- MCAD Deficiency.- D-Hydroxyacyl-CoA Dehydrogenase Deficiency.- Phytanoyl-CoA Hydroxylase Deficiency.- Rationale for a Conditional Knockout Mouse Model to Study Carnitine Palmitoyltransferase I Deficiencies.- Biochemical Characterisation of Mutations of Human Medium-Chain Acyl-CoA Dehydrogenase.- Lessons Learned from The Mouse Model of Short-Chain Acyl-CoA Dehydrogenase Deficiency.