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Previous Volume 348:2656-2668 June 26, 2003 Number 26 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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More than a billion years ago, aerobic bacteria colonized primordial eukaryotic cells that lacked the ability to use oxygen metabolically. A symbiotic relationship developed and became permanent. The bacteria evolved into mitochondria, thus endowing the host cells with aerobic metabolism, a much more efficient way to produce energy than anaerobic glycolysis. Structurally, mitochondria have four compartments: the outer membrane, the inner membrane, the intermembrane space, and the matrix (the region inside the inner membrane). They perform numerous tasks, such as pyruvate oxidation, the Krebs cycle, and metabolism of amino acids, fatty acids, 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.

Related Letters:

Mitochondrial Diseases
Fischel-Ghodsian N., DiMauro S., Schon E. A.
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N Engl J Med 2003; 349:1293-1294, Sep 25, 2003. Correspondence

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