HTT and Huntington disease: Large neuronal intranuclear inclusion bodies (IBs) that contain mutant HTT (mHTT) protein aggregates, molecular chaperones and ubiquitin are the prominent hallmark of the pathology in HD patient brains (DiFiglia et al., 1997; Sieradzan et al., 1999), suggesting that misfolding and abnormal aggregation of mHTT may contribute to pathogenesis or even drive the disease process (Wanker et al., 2019).