Current Views of Fatty Acid Oxidation and Ketogenesis : From Organelles to Point Mutations - Quant, Patti A.; Eaton, Simon; SpringerLink (Online service) (New York, NY : Springer US : Imprint: Springer, 2002)
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 from Abnormal 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 with Lethal 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.
Keywords: Biochemistry; Internal medicine; Nutrition .; Biochemistry, general; Internal Medicine; Nutrition