Neuronal cell death in Huntington's disease: a potential role for dopamine

Huntington's disease is an inherited neurodegenerative disorder, the cause of which is unknown. Excitotoxicity, mitochondrial dysfunction and oxidativ e stress are all likely to contribute to the striatal cell death that occur s in this disorder. There are accumulating data indicating that under speci fic circumstances, dopamine, which occurs in high concentrations in the bas al ganglia, might be neurotoxic. In this article, the current models used t o study Huntington's disease are reviewed and the recent findings that impl icate dopamine in the pathophysiology of this progressive disorder are disc ussed. Although many questions remain unanswered, the dopaminergic system c ould contribute to striatal vulnerability in Huntington's disease and provi de a novel avenue for the development of new therapies.