Neurodegeneration in Huntington's disease involves loss of cystathionine γ-lyase

摘要

Huntington's disease (HD) is an auto-somal dominant lethal neurologic disorder. Its pathophysiology involves massive, highly selective degeneration of the corpus striatum of the brain, which regulates motor activity, and whose loss in HD leads to the characteristic choreiform movements of patients. The unique degradation of the striatum appears to reflect the binding of mutant huntingtin to a striatal-selective small G protein called Rhes. Recendy, we have identified specific molecular mechanisms whereby huntingtin elicits neuronal destruction.