More than a billion years ago, aerobic bacteria colonized primordialeukaryotic cells that lacked the ability to use oxygen metabolically.A symbiotic relationship developed and became permanent. Thebacteria evolved into mitochondria, thus endowing the host cellswith aerobic metabolism, a much more efficient way to produceenergy than anaerobic glycolysis. Structurally, mitochondriahave four compartments: the outer membrane, the inner membrane,the intermembrane space, and the matrix (the region inside theinner membrane). They perform numerous tasks, such as pyruvateoxidation, the Krebs cycle, and metabolism of amino acids, fattyacids, and steroids, but the most crucial is probably . . . [Full Text of this Article]
Mitochondrial Genetics
Maternal Inheritance
Heteroplasmy and the Threshold Effect
Mitotic Segregation
Respiratory-Chain Disorders Due to Defects in mtDNA
Respiratory-Chain Disorders Due to Defects in nDNA
Mutations in Structural Components of the Respiratory Chain
Mutations in Ancillary Proteins of the Respiratory Chain
Defects in Intergenomic Signaling Affecting Respiratory Function
Defects of the Membrane Lipid Milieu
Disorders with Indirect Involvement of the Respiratory Chain
Defects of Mitochondrial Protein Importation
Defects in Mitochondrial Motility
Neurodegenerative Diseases
Therapeutic Approaches
Source Information
From the Departments of Neurology (S.D., E.A.S.) and Genetics and Development (E.A.S.), Columbia University College of Physicians and Surgeons, New York.
Address reprint requests to Dr. DiMauro at 4-420 College of Physicians and Surgeons, 630 W. 168th St., New York, NY 10032, or at sd12@columbia.edu.
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