PDK1 and Huntington disease: In summary, our study shows in large detail mitochondrial impairment that could be attributed to reduced mitochondrial biogenesis and decreased CxIII and PDK1 levels, causing metabolic imbalance, and enhanced mitochondrial generation of ROS, linked to round shape mitochondrial morphology in early stages of undifferentiation, in human HD iPSC, which were mostly replicated in iPSC-derived NSC expressing mHTT.