Table 1. Components of nuclear protein inclusions with listing of their histochemical and biophysical amyloid features.
Amyloid component | IHC | NI | PolyQ- induced |
PolyQ disease |
Animal model |
Cell culture |
CR/ThT | CR bi | AP | anti Aβ | Reference |
---|---|---|---|---|---|---|---|---|---|---|---|
huntingtin | ● | ● | ● | ● | ● | ● | ● | ● | 31, 32, 71, 72 | ||
ubiquitin | ● | ● | ● | ● | ● | ● | 31, 32, 43, 72 | ||||
androgen receptor | ● | ● | ● | ● | ● | ● | 36, 73 | ||||
ataxin-1 | ● | ● | ● | ● | ● | ● | 38, 74 | ||||
20S proteasome | ● | ● | ● | ● | ● | 38, 41 | |||||
HDJ-2/HSDJ | ● | ● | ● | ● | ● | 38 | |||||
HSP-70 | ● | ● | ● | ● | ● | 38, 43, 75 | |||||
p53 | ● | ● | ● | 43 | |||||||
mdm-2 | ● | ● | ● | 43 | |||||||
TATA binding protein | ● | ● | ● | ● | ● | ● | 43, 72, 76, 77 | ||||
NPC proteins | ● | ● | ● | ● | 43 | ||||||
ataxin-3 | ● | ● | ● | ● | ● | 78, 79 | |||||
atrophin-1 | ● | ● | ● | ● | 80 | ||||||
CREB binding protein | ● | ● | ● | ● | ● | 45, 80 | |||||
PML | ● | ● | ● | ● | ● | ● | 34, 74, 80 | ||||
SUMO-1 | ● | ● | ● | ● | ● | ● | 34, 81 | ||||
SUMO-2 | ● | ● | ● | ● | ● | ● | 81 | ||||
ataxin-7 | ● | ● | ● | ● | ● | ● | 81 | ||||
p80 coilin | ● | ● | ● | ● | ● | 34, 74 | |||||
histone H3 | ● | ● | ● | ● | 82 | ||||||
hnRNP F/H | ● | ● | ● | ● | 82 | ||||||
smn1 | ● | ● | ● | ● | 15 | ||||||
gemin4 | ● | ● | ● | ● | 15 | ||||||
SR140 | ● | ● | ● | ● | 15 | ||||||
WDR3 | ● | ● | ● | ● | 15 | ||||||
ranBP1 | ● | ● | ● | ● | 15 | ||||||
laminB1 | ● | ● | ● | ● | 15 | ||||||
NuMA | ● | ● | ● | ● | 15 | ||||||
nuclear amyloid structure | ● | ● | ● | ● | ● | ● | ● | 24, 25, 40 |
Anti Aβ, detection by amyloid specific antibodies; AP, amyloid peptide binding; CR bi, Congo red birefringence; CR/ThT, Congo red and/or Thioflavin T binding; IHC, nuclear inclusions defined by immunohistochemistry; NI, nuclear inclusion.