Abstract: Huntington’s disease (HD) (OMIM 143100) is an autosomal dominant neurodegenerative disorder caused by a monogenic mutation in the huntingtin gene (HTT), which induces typical midlife onset and age-dependent progression with major symptoms including choreic movements, psychiatric disorders, and cognitive impairment (Gusella et al., 2021). After the 1993 discovery of a pathogenic expansion of the CAG trinucleotide repeat beyond 35 in HTT exon 1 as a causative factor for HD, many animal, mammalian cell and yeast models expressing mutant HTT (mHtt) with abnormal CAG repeats have been created to study CAG repeat-induced toxicity (Naphade et al., 2019; Gusella et al., 2021). With these models and studies on human subjects, some important insights into disease initiation and progression mechanisms have been elucidated, but the underlying mHtt-induced toxicity mechanisms are still not yet fully understood with no disease-modifying treatment in sight (Caron et al., 2018; Veldman and Yang, 2018; Gusella et al., 2021).