Impairment of energy metabolism and excitotoxic cell death in Huntington disease.
The gene for Huntington's disease is widely expressed in brain and yet the illness is characterized pathologically by a distinct regional pattern of cell death. Various theories for this selective vulnerability have been offered but the most compelling remains that of abnormal energy metabolism. These issues are reviewed.
Massachusetts General Hospital, Boston 02114, USA.
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Young
D017209:Apoptosis / Q000502:physiology*; D004734:Energy Metabolism; D006801:Humans; D006816:Huntington Disease / Q000235:genetics / Q000378:metabolism*; D009474:Neurons / Q000378:metabolism*