Many human genetic diseases associated with blood, brain, colon, ear, eye, heart, kidney, liver, muscle, and pancreas are caused by mutations in mitochondrial DNA. Mutations in DNA can result in defects of the electron transport complexes, intermediates of the tricarboxylic acid cycle and substrate transport. The clinical manifestation of these diseases often involves muscle and the nervous system. Mitochondrial DNA mutations have now been associated with aging as well as age-related degenerative diseases such as Parkinson's, Alzheimer's, and Huntington's diseases. Changes in structure, function, and a number of mitochondria play an important role in carcinogenesis. Furthermore, the role of mitochondria in the execution of programmed cell death or apoptosis has been recognized recently.

1. Introduction

2. Mitochondrial Structure, Function and Biogenesis
3. The Mitochondrial Genetic System
4. Inheritance of Mitochondrial Mutations
5. Mitochondrial DNA Replication
6. Genetic Integrity of the Mitochondrial Genome
7. Modeling Mitochondrial DNA Mutations
8. Mitochondrial Regulation of Apoptosis
9. Characteristics of Mitochondrial DNA Diseases
10. Nuclear Defects Affecting Mitochondrial Function
11. Mitochondrial DNA Mutations in Aging
12. Mitochondrial DNA Mutations and Heart Disease
13. Mitochondrial Dysfunction and Neurodegenerative Diseases
14. Toxin Induced Mitochondrial Dysfunction and Neurodegeneration
15. Perspectives on Mitochondria in Carcinogenesis
16. The Mitochondrion as a Target for Cancer Chemotherapy
17. Prohibitin: Mitochondrial Tumor Suppressor Protein
18. Abnormal Growth and Male Sterility Associated with Mitochondrial DNA Rearrangements in Plants
19. Mitochondrial Disorder and Migraine
20. Gene Therapy of Mitochondrial DNA Diseases
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