Supplementary Figure 8: A model for the mutant huntingtin-induced mitochondrial protein import defect.
From: Inhibition of mitochondrial protein import by mutant huntingtin
Mutant Htt binds the TIM23 complex and prevents the import of nuclear-encoded proteins into neuronal mitochondria early in HD pathogenesis, causing mitochondrial dysfunction and subsequent neuronal dysfunction and death in HD. Inhibition of mitochondrial protein import in R6/2 mice is tissue- and age-dependent.
