Mihondria 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 mihondrial 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 mihondria in different organisms. It particularly focuses on the recent advances in our understanding of basic mihondrial research to the consequences of dysfunction at the molecular level. The 13 contributions written by leading researchers in the field include topics such as: mihondrial genome evolution and mtDNA stability, mihondrial biogenesis and protein quality control, mihondrial morphology, assembly and function of the mihondrial energy generation apparatus and mihondrial metabolic pathways. These are particularly oriented to link in these various mihondrial pathways to the clinical consequences of their dysfunctions.