Figure 1.
Functional alterations in HD mitochondria. The cartoon depicts the functional alterations occurring at the level of mitochondrial function, opening of the PTP, and morphology in the context of HD. Mutated Huntingtin can act at the transcriptional level, by inhibiting PGC1α, directly at the mitochondrial level, by increasing the open probability of the PTP and affecting mitochondrial respiratory chain, and at the level of DRP1 causing fragmentation and remodelling of the mitochondrial cristae.