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.
Young
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AB
Massachusetts General Hospital, Boston 02114, USA.
D017209:Apoptosis / Q000502:physiology*; D004734:Energy Metabolism; D006801:Humans; D006816:Huntington Disease / Q000235:genetics / Q000378:metabolism*; D009474:Neurons / Q000378:metabolism*