Oxidative Phosphorylation in Health and Disease [Paperback]

Mitochondrial diseases are often hard to diagnose. From the time they were first researched without animal models, patients of mitochondrial diseases were of equal interest to both clinical and basic scientists. With the new research done, this book includes updates on the normal structure, function, and molecular biology of the mitochondrial respiratory chain, information on traditional diagnostical methodologies, and an overview of the diagnostic promise of new technologies. The hypermetabolism of Luft disease, although only seen twice, is also studied. There are critical reviews of symptoms and signs associated with syndromes, as well as updates on the genetic defects of either the mitochondrial or the nuclear genome responsible for many disorders.Mitochondrial diseases are often hard to diagnose. From the time they were first researched without animal models, patients of mitochondrial diseases were of equal interest to both clinical and basic scientists. With the new research done, this book includes updates on the normal structure, function, and molecular biology of the mitochondrial respiratory chain, information on traditional diagnostical methodologies, and an overview of the diagnostic promise of new technologies. The hypermetabolism of Luft disease, although only seen twice, is also studied. There are critical reviews of symptoms and signs associated with syndromes, as well as updates on the genetic defects of either the mitochondrial or the nuclear genome responsible for many disorders.CONTENTS Foreword 1.The Human OXPHOS System: Structure,Function and Physiology; Immo E.Scheffler.- Complexes of the Electron Transport Chain.- The ATP Synthase.- Regulation of Oxidative Phosphorylation.- Assembly of Electron Transport Complexes.- 2.Molecular Biology of the OXPHOS System; Richard C.S carpulla.- mtDNA.- Mitochondrial Inheritance.- Replication,Transcription,RNA Processing.- Recombination and Repair.- Mitochondrial Translation System.- Bi-Genomic Expression of lc$