Presenting both historical and recent research in all aspects of fatty acid oxidation, this book forms a unique synthesis of information concerning normal and abnormal fatty acid oxidation that includes data from clinical, biochemical, and molecular studies. Fatty Acid Oxidation features a discussion of the reaction mechanisms of acyl-CoA dehydrogenases, and an examination of acyl-CoA dehydrogenase deficiencies and their relationship to sudden infant death syndrome. This diversified book also reviews diagnostic methods for identifying patients with acyl-CoA dehydrogenase defects and covers disorders of mitochondrial and peroxisomal fatty acid oxidation. Descriptions of the molecular aspects of fatty acid oxidation enzymes and studies of the molecular basis of isovaleric acidemia are also included. Describes appropriate diagnostic methods for identifying patients with metabolic disorders before they become symptomatic.
|Series:||Progress in Clinical and Biological Research Series , #54|
|Product dimensions:||1.38(w) x 2.17(h) x (d)|
Table of Contents
Partial table of contents:
Fatty Acid Oxidation in Soluble Systems of Mammalian Origin: The Beginnings (H. Beinert).
ENZYMES OF β-OXIDATION.
The Reductive Half Reaction in the Acyl-CoA Dehydrogenases (C. Thorpe).
INHIBITORS OF β-OXIDATION.
Mechanisms of Enzyme Inhibition by Hypoglycin, Methylenecyclopropylglycine, and Their Metabolites (S. Ghisla, et al.).
Riboflavin Deficiency (C. Hoppel & B. Tandler).
ACYL-CoA DEHYDROGENASE DEFICIENCIES.
Carnitine Homeostasis in the Organic Acidurias (C. Roe, et al.).
OTHER FATTY ACID OXIDATION DEFECTS.
A Genetic Defect in Carnitine Transport Causing Primary Carnitine Deficiency (C. Stanley, et al.).
MOLECULAR ASPECTS OF FATTY ACID OXIDATION ENZYMES.
Biosynthesis, Molecular Cloning, and Sequencing of Electron Transfer Flavoprotein (G. Finocchiaro, et al.).