Mitochondria: The Next (Neurode)Generation

被引:448
作者
Schon, Eric A. [1 ,2 ,4 ,5 ]
Przedborski, Serge [1 ,3 ,4 ]
机构
[1] Columbia Univ, Dept Neurol, New York, NY 10032 USA
[2] Columbia Univ, Dept Genet & Dev, New York, NY 10032 USA
[3] Columbia Univ, Dept Pathol & Cell Biol, New York, NY 10032 USA
[4] Columbia Univ, Ctr Motor Neuron Biol & Dis, New York, NY 10032 USA
[5] Columbia Univ, H Houston Merritt Clin Res Ctr, New York, NY 10032 USA
基金
美国国家卫生研究院;
关键词
MARIE-TOOTH-DISEASE; HEREDITARY SPASTIC PARAPLEGIA; AMYOTROPHIC-LATERAL-SCLEROSIS; M-AAA PROTEASE; SUBSTANTIA-NIGRA NEURONS; KINESIN HEAVY-CHAIN; ALZHEIMERS-DISEASE; ENDOPLASMIC-RETICULUM; PARKINSONS-DISEASE; MUTANT HUNTINGTIN;
D O I
10.1016/j.neuron.2011.06.003
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
Adult-onset neurodegenerative disorders are disabling and often fatal diseases of the nervous system whose underlying mechanisms of cell death remain unknown. Defects in mitochondrial respiration had previously been proposed to contribute to the occurrence of many, if not all, of the most common neurodegenerative disorders. However, the discovery of genes mutated in hereditary forms of these enigmatic diseases has additionally suggested defects in mitochondria! dynamics. Such disturbances can lead to changes in mitochondrial trafficking, in interorganellar communication, and in mitochondrial quality control. These new mechanisms by which mitochondria may also be linked to neurodegeneration will likely have far-reaching implications for our understanding of the pathophysiology and treatment of adult-onset neurodegenerative disorders.
引用
收藏
页码:1033 / 1053
页数:21
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