Department of Neurology, University of Kansas School of Medicine, Kansas City, KS 66209, USA.
Adv Exp Med Biol. 2012;942:269-86. doi: 10.1007/978-94-007-2869-1_12.
Many neurodegenerative diseases demonstrate abnormal mitochondrial morphology and biochemical dysfunction. Alterations are often systemic rather than brain-limited. Mitochondrial dysfunction may arise as a consequence of abnormal mitochondrial DNA, mutated nuclear proteins that interact directly or indirectly with mitochondria, or through unknown causes. In most cases it is unclear where mitochondria sit in relation to the overall disease cascades that ultimately causes neuronal dysfunction and death, and there is still controversy regarding the question of whether mitochondrial dysfunction is a necessary step in neurodegeneration. In this chapter we highlight and catalogue mitochondrial perturbations in some of the major neurodegenerative diseases including Alzheimer's disease (AD), Parkinson's disease (PD), amyotrophic lateral sclerosis (ALS), and Huntington's disease (HD). We consider data that suggest mitochondria may be critically involved in neurodegenerative disease neurodegeneration cascades.
许多神经退行性疾病表现出异常的线粒体形态和生化功能障碍。这些改变往往是全身性的，而不仅仅局限于大脑。线粒体功能障碍可能是由于异常的线粒体 DNA、直接或间接与线粒体相互作用的突变核蛋白，或由于未知原因引起的。在大多数情况下，尚不清楚线粒体在最终导致神经元功能障碍和死亡的整体疾病级联反应中处于什么位置，并且对于线粒体功能障碍是否是神经退行性变的必要步骤仍存在争议。在本章中，我们重点介绍并分类了一些主要神经退行性疾病中的线粒体扰动，包括阿尔茨海默病 (AD)、帕金森病 (PD)、肌萎缩侧索硬化症 (ALS) 和亨廷顿病 (HD)。我们考虑了一些数据，这些数据表明线粒体可能在神经退行性疾病的神经退行性级联反应中起着至关重要的作用。
