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Huntington's Disease: the mystery unfolds?

Petersén Å and Brundin P.

International Review of Neurobiology 53: 315-339 (2002) 


The Huntington's disease (HD) has stimulated novel clinical drug trials based on the ideas about mechanisms of cell death in the disorder. A recent study investigated the effects of remacemide and coenzyme Q on progression of symptoms in HD. These drugs are an N-methyl-d-aspartate (NMDA) antagonist and a mitochondrial electron transfer co-factor, respectively, and were chosen based on the theories that excitotoxicity and energy impairment play roles in the disease. Neither drug was effective, but the trial represents the opening of a new era of trials based on rational theories derived from experimental work in cultures and animals. The presence of neuronal intranuclear inclusions in symptomatic HD patients, and their absence in presymptomatic patients, suggests that they are closely linked to the onset of the disease. Despite several important findings regarding the pathogenesis of HD, several fundamental issues remain to be resolved. For example, mechanisms underlie the correlation between CAG-repeat length and age of onset, and the other factors influence the appearance of symptoms.