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Table 2 Proteins and domains that facilitate LLPS in NDDs

From: Stress granules: emerging players in neurodegenerative diseases

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]

  1. 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