Table 2.

Proteins and domains that facilitate LLPS in NDDs

Names of proteins	RNA-binding domain			LLPS-promoting domains				References
ATXN2	LSm	LSmAD		Poly Q (548–571)	Poly Q (616–656)	cIDR (900–1084)	–	[88, 148]
FUS	RRM	–	–	PrLD	RGG (371–422)	RGG (453–501)	PY-NLS (501–526)	[149]
G3BP1/2	RRM	–	–	NTF2L	Gly-rich	–	RGG	[34, 90]
hnRNPA1	RRM1	RRM2	–	LCD	–	–	–	[92, 150]
hnRNPA2/B2	RRM1	RRM2	–	LCD				[150]
Tau	–	–	–	PRD (P1, P2)	MTBD (R1, R2, R3, R4)	–	–	[82, 151]
TDP-43	RRM1	RRM2	–	LCD	–	–	–	[149]
TIA1	RRM1	RRM2	RRM3	LCD	–	–	–	[104]

ATXNT2, ataxin-2; FUS, fused in sarcoma; cIDR, C-terminal intrinsically disordered region; G3BP1/2, Ras-GAP SH3-domain-binding protein 1/2; Gly-rich, Glycine rich region; hnRNPA1, heterogeneous nuclear ribonucleoprotein A1; hnRNPA2/B1, heterogeneous nuclear ribonucleoprotein; LCD, low complexity domain; LSm, like-Sm; LSmAD, like-Sm associated domain; MTBD, microtubule-binding domain; NTF2L, nuclear transport factor 2-like; PolyQ, Polyglutamine; PRD (P1/2), Proline-rich domain 1/2; PrLD, Prion-like Domain; PY-NLS, PY-Nuclear localization signals; RGG, arginine‐glycine‐glycine repeat; RRM, RNA recognition motif; TDP-43, TAR DNA-binding protein 43; TIA1, T cell-restricted intracellular antigen-1