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. 2004 Sep;24(18):8195–8209. doi: 10.1128/MCB.24.18.8195-8209.2004

FIG. 8.

FIG. 8.

Proposed mechanism of neuronal dysfunction in HD. Model for HD pathogenesis that includes inhibition of mhtt proteolysis and initiation of aggregation that involves specific cellular targets along with wild-type htt. Sequestration leads to disruption of cytoskeleton, vesicular and organelle trafficking, transcriptional dysfunction, and eventually causes mitochondrial dysfunction and neuronal death.