Figure 1. Schematic representation of proteins in nuclear inclusions (NIs) from patients with neurodegenerative polyQ (CAG) expansion diseases and respective animal or mammalian cell culture models. 20S, 20S proteasome subunits; 19S, 19S proteasome regulator subunits; 11S, 11S proteasome regulator subunits; AR, androgen receptor; atxn-1, ataxin-1; atxn-3, ataxin-3; atxn-7, ataxin-7; CBP, CREB binding protein; EYA, eyes absent protein; hnRNPs, heterogeneous nuclear ribonucleoproteins; HSDJ, DNAJ protein homolog; HSP-70, heat shock protein 70; htt, huntingtin; mdm-2, mouse double minute protein; NP, nanoparticle; NPC, nuclear pore complex; NuMA, nuclear mitotic apparatus protein; PML, promyelocytic leukemia protein; Qn, different length polyQ repeat; SUMO, small ubiquitin-related modifier; ub, ubiquitinated; TBP, TATA binding protein.