Huntington's disease and mitochondrial alterations: emphasis on experimental models.

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.