Table of Contents

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.

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