The Biology of Huntington’s Chorea

  • Ara Tourian


This chapter is an overview of the analysis provided by the diverse disciplines concerned with the biology of Huntington’s chorea, which is documented in a vast literature. It is not a comprehensive treatment, because excellent reviews (some of which are cited) cover many of the individual areas. This review has two goals: (1) to bring into sharp focus those observations that have endured the test of time and shed light on the more general features of the correlations among the clinical, neuropathological, and neurochemical-neuropathological observations of Huntington’s chorea, and (2) to call attention to a newer approach—that of cell biology and nutrition of peripheral tissues such as skin fibroblasts—to the analysis of dominantly inherited brain disease. The insights generated by such a strategy regarding potential metabolic pathway abnormalities should be directly testable in HC brain. Ultimately, this type of approach may help solve the fundamental biochemical genetic problem of HC and serve as a model for unraveling the primary metabolic abnormalities of other dominantly inherited diseases.


Minimal Essential Medium Kainic Acid Myotonic Dystrophy Pathological Neurochemistry Acute Intermittent Porphyria 
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Copyright information

© Plenum Press, New York 1985

Authors and Affiliations

  • Ara Tourian
    • 1
  1. 1.Neurogenetics and Cell Biology Laboratory, Department of Medicine, Division of NeurologyDuke University Medical CenterDurhamUSA

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