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. Author manuscript; available in PMC: 2015 May 29.
Published in final edited form as: Nat Neurosci. 2014 Jun;17(6):747–749. doi: 10.1038/nn.3726

Figure 1.

Figure 1

Mutant huntingtin inhibits mitochondrial protein import. Shown are cytosolic compartment, mitochondrial inner and outer membranes, intermembrane space and mitochondrial matrix. Mitochondrial preproteins translocate through the TOM complex in the outer membrane. Matrix and many inner membrane and intermembrane proteins are recognized by TIM23 subunits and translocate through the TIM23 complex. Mutant huntingtin full-length protein or mutant huntingtin N-terminal peptide, both containing long polyglutamine (polyQ) repeat sequences, bind the TIM23 complex, inhibiting protein import.