Laboratorio de Aminoácidos Excitadores, Instituto Nacional de Neurología y Neurocirugía, Mexico City, 14269, Mexico.
J Bioenerg Biomembr. 2010 Jun;42(3):207-15. doi: 10.1007/s10863-010-9289-4.
Huntington's disease (HD) is an inheritable neurological disorder coursing with degeneration of basal ganglia and producing chorea and dementia. One common factor accounting for neurodegeneration in this disorder is mitochondrial deterioration at both morphologic and functional levels. The development of experimental models in animals or cell preparations to resemble pathologic and pathogenic conditions of this disorder has served for more than four decades to describe part of the mechanistic alterations that could be occurring in mitochondria of HD patients, and the subsequent design of therapeutic alternatives where mitochondrial alterations are the primary target. In this minireview we describe some of the most relevant studies at the experimental level, giving support to the hypothesis that mitochondria play a central role in HD pathogenesis.
亨廷顿病（HD）是一种遗传性神经退行性疾病，伴有基底神经节的退化，导致舞蹈病和痴呆。这种疾病中神经退行性变的一个共同因素是形态和功能水平的线粒体恶化。在动物或细胞制剂中开发类似该疾病的病理和发病条件的实验模型，四十多年来一直用于描述可能发生在 HD 患者线粒体中的部分机制改变，以及随后设计以线粒体改变为主要靶点的治疗替代方案。在这篇综述中，我们描述了一些在实验水平上的相关研究，为线粒体在 HD 发病机制中起核心作用的假说提供了支持。
