Mitochondria are complex organelles, possessing a double-membrane and even their own genome, the mtDNA. They play a pivotal role in cellular metabolism, respiration, and production of ATP essential for the normal function of all human organ systems. It is not surprising, therefore, that genetic defects of mitochondrial functions cause a wide spectrum of human diseases. This book provides the first modern and truly comprehensive coverage of the biochemistry, genetics, and pathology of mitochondria in different organisms. It particularly focuses on the recent advances in our understanding of basic mitochondrial research to the consequences of dysfunction at the molecular level. The 13 contributions written by leading researchers in the field include topics such as: mitochondrial genome evolution and mtDNA stability, mitochondrial biogenesis and protein quality control, mitochondrial morphology, assembly and function of the mitochondrial energy generation apparatus and mitochondrial metabolic pathways. These are particularly oriented to link in these various mitochondrial pathways to the clinical consequences of their dysfunctions.
Mitochondrial Genome Evolution: The Origin of Mitochondria and of Eukaryotes * Mitochondrial Biogenesis: Protein Import into and Across the Outer Membrane * Mitochondrial Biogenesis. Protein Import into and Across the Inner membrane * Mitochondrial tRNA Editing * Protein Quality Control in Mitochondria and Neurodegeneration in Hereditary Spastic Paraplegia * Defects in Assembly of Cytochrome Oxidase: Roles in Mitochondrial Disease * Function and Dysfunction of the Oxidative Phosphorylation System * mtDNA Maintenance and Stability Genes: MNGIE and mtDNA Depletion Syndromes * Protein Translocation into Mammalian Mitochondria and Its Role in the Development of Human Mitochondrial Disorders * Mitochondrial Fission and Fusion Machineries * VDAC Function in a Cellular Context * Lipid Synthesis and Transport in Mitochondrial Biogenesis * From Electron Transfer to Cholesterol Transfer; Molecular Regulation of Steroid Synthesis in the Mitochondrion.