Current Views of Fatty Acid Oxidation and Ketogenesis: From Organelles to Point Mutations

by Patti A. Quant (Editor), Simon Eaton (Editor)

Overview

This book brings together biochemical, genetic, molecular biology, and clinical approaches to the study of fatty acid oxidation. Including current research from most of the major groups working in this field, it provides a multi-disciplinary approach to the subject and an up-to-date overview of the most recent developments and debates.

Product Details

ISBN-13:	9781475772111
Publisher:	Springer US
Publication date:	04/26/2013
Series:	Advances in Experimental Medicine and Biology , #466
Edition description:	2002
Pages:	414
Product dimensions:	6.10(w) x 9.25(h) x 0.04(d)

Table of Contents

Biogenesis of the Rat Liver Mihondrial Carnitine Palmitoyltransferase I.- Subcellular Distributuon of Mihondrial Carnitine Palmitoyltransferase I in Rat Liver.- Topology of Hepatic Mihondrial 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 Hepaytes.- 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 Mihondria.- 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 Mihondrial—-Oxidation at the Levels of [NAD+]/[NADH] and CoA Acylation.- Production and Export of Acylcarnitine Esters by Neonatal Rat Hepaytes.- Tissue Specific Differences in Intramihondrial 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 Mihondrial 3-Hydroxy-3-Methylglutaryl-CoA Synthase.- Regulation of the Ketogenic Enzyme Mihondrial 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.