Current Views of Fatty Acid Oxidation and Ketogenesis: From Organelles to Point Mutations: Advances in Experimental Medicine and Biology, cartea 466

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 a“lipostat” 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.