Mitochondrial Diseases in Man and Mouse

Over the past 10 years, mitochondrial defects have been implicated in a wide variety of degenerative diseases, aging, and cancer. Studies on patients with these diseases have revealed much about the complexities of mitochondrial genetics, which involves an interplay between mutations in the mitochon...

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Bibliographic Details
Published in:Science (American Association for the Advancement of Science) 1999-03, Vol.283 (5407), p.1482-1488
Main Author: Wallace, Douglas C.
Format: Article
Language:English
Subjects:
Aging - genetics
Animals
Apoptosis
Cell lines
Cellular biology
Computer Peripherals
Disease
DNA, Mitochondrial - genetics
Energy
Genetic mutation
Genetics
Humans
Medical genetics
Metabolic Diseases - genetics
Metabolic Diseases - metabolism
Metabolism
Mice
Mitochondria
Mitochondria - genetics
Mitochondria - metabolism
Mitochondrial diseases
Mitochondrial DNA
Mitochondrial myopathies
Mitochondrial Myopathies - genetics
Mitochondrial Myopathies - metabolism
Mutation
Neoplasms - genetics
Neoplasms - metabolism
Nervous system diseases
Oxidative Phosphorylation
Pathology
Reviews
Rodents
Skeletal muscle
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Summary:Over the past 10 years, mitochondrial defects have been implicated in a wide variety of degenerative diseases, aging, and cancer. Studies on patients with these diseases have revealed much about the complexities of mitochondrial genetics, which involves an interplay between mutations in the mitochondrial and nuclear genomes. However, the pathophysiology of mitochondrial diseases has remained perplexing. The essential role of mitochondrial oxidative phosphorylation in cellular energy production, the generation of reactive oxygen species, and the initiation of apoptosis has suggested a number of novel mechanisms for mitochondrial pathology. The importance and interrelationship of these functions are now being studied in mouse models of mitochondrial disease.
ISSN:0036-8075
1095-9203
DOI:10.1126/science.283.5407.1482