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Inborn and Induced Defects of Mitochondria
Anthony H. V. Schapira, DSc, MD, FRCP
Arch Neurol. 1998;55:1293-1296.
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INTRODUCTION
Mitochondria play a pivotal role in cellular metabolism and in energy production in particular. Predictably, defects of mitochondrial metabolism have a deleterious effect on cell function and survival, especially in highly energy-dependent tissues such as brain and skeletal muscle. Although a multitude of biochemical reactions occur within mitochondria, the oxidative phosphorylation (OXPHOS) system is the most important in terms of adenosine triphosphate generation and in its association with human disease.
The OXPHOS system is located on the inner mitochondrial membrane and comprises the respiratory chain (complexes I-IV) and adenosine triphosphate synthase (complex V). It is responsible for proton pumping, producing the transmembranous electrochemical gradient (  m), and generating adenosine triphosphate by aerobic metabolism. The 5 protein complexes of the OXPHOS system comprise approximately 82 subunits, 13 of which are encoded by mitochondrial DNA (mtDNA). Human mtDNA is a 16.5-kilobase circular double-stranded molecule that . . . [Full Text of this Article]
PRIMARY DEFECTS: mtDNA
PRIMARY DEFECTS: NUCLEAR DNA
MITOCHONDRIAL DYSFUNCTION IN NEURODEGENERATION
Parkinson Disease Huntington Disease Friedreich Ataxia Alzheimer Disease Dystonia
NEURODEGENERATION: CONCLUSIONS
From the University Department of Clinical Neurosciences, Royal Free Hospital School of Medicine, and University Department of Clinical Neurology, Institute of Neurology, London, England.
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ABSTRACT
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