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Review
. 2009;17(4):737-51.
doi: 10.3233/JAD-2009-1095.

The neurodegenerative mitochondriopathies

Russell H Swerdlow  1
Affiliations
Review

The neurodegenerative mitochondriopathies

Russell H Swerdlow. J Alzheimers Dis. 2009.

Abstract

Mitochondria are physically or functionally altered in many neurodegenerative diseases. This is the case for very rare neurodegenerative disorders as well as extremely common age-related ones such as Alzheimer's disease and Parkinson's disease. In some disorders very specific patterns of altered mitochondrial function or systemic mitochondrial dysfunction are demonstrable. Some disorders arise from mitochondrial DNA mutation, some from nuclear gene mutation, and for some the etiology is not definitively known. This review classifies neurodegenerative diseases using mitochondrial dysfunction as a unifying feature, and in doing so defines a group of disorders called the neurodegenerative mitochondriopathies. It discusses what mitochondrial abnormalities have been identified in various neurodegenerative diseases, what is currently known about the mitochondria-neurodegeneration nexus, and speculates on the significance of mitochondrial function in some disorders not classically thought of as mitochondriopathies.

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Figures

Figure 1
Figure 1
The cybrid technique. The black-filled circles represent nuclei. The ovals represent mitochondria. The circles within the ovals represent mitochondrial DNA, with different patterns indicating mtDNA from different individuals.
Figure 2
Figure 2
The Alzheimer’s disease mitochondrial cascade hypothesis.
See this image and copyright information in PMC

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