HTT and juvenile Huntington disease: Huntington’s disease (HD) is an autosomal dominant disorder caused by a polyglutamine repeat expansion in the N-terminus of the huntingtin protein (Htt) that results in myriad molecular and pathophysiological features, including mitochondrial dysfunction, intracellular trafficking defects, abnormalities of protein folding and/or processing, and the diagnostic motor and cognitive defects [1], [2].  Gross defects like huntingtin aggregation and nuclear accumulation are thought to represent intermediate to advanced stages of molecular pathology[3] .