Mitochondrial dysfunction associated with dysregulation of AMPK- SIRT1 (Sirtuin 1)- NRF1/2 (nuclear respiratory factor 1/2)—TFAM (Mitochondrial transcription factor A) axis has been implicated in several neuronal diseases, which include Alzheimer’s disease, parkinsonism, diabetic neuropathy, nerve injury, Huntington’s disease, and PIPN [23,24,25]. Here, NRF1 is linked to juvenile Huntington disease.