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.