Table 2.

Characteristics of Key RNA Binding Proteins That Localize to Stress Granules

RBP name	IDR/LCD	Prion-like	Self- interaction	Interactions with other SG RBPs	Disruption decreases SGs?	SGs form with overexpression?	Associated genetic diseases	Identified roles in translation	References
G3BP1/2	+	−	Dimer	UBAP2/2L, Caprin	++	Yes	Neurodevelopmental disease	Promotes translation via ribosome recycling in unstressed cells	Huang et al. (2020), Jia et al. (2022); Meyer et al. (2020); Reineke et al. (2012); Yang et al. (2020)
UBAP2/2L	+	−	Dimer	G3BP1/2, FMRs/FXRs	++	Yes	Neurodevelopmental disease	Promotes translation of bound mRNAs in unstressed cells	Cirillo et al. (2020); Huang et al. (2020); Jia et al. (2022); Luo et al. (2020)
TIA-1/TIAR	+	+	Oligomer	n/a	+	Yes	ALS, FTD, Welander distal myopathy	Inhibits translation in unstressed cells, regulates mRNAs with 5′ terminal oligopyrimidine tracts	Damgaard and Lykke-Andersen (2011); Gilks et al. (2004); Hackman et al. (2013); Hirsch-Reinhagen et al. (2017); Waris et al. (2014)
hnRNPA1	+	+	Oligomer	n/a	−/+	No	ALS, FTD, multisystem proteinopathy, multiple hereditary neuropathies, and myopathies	Regulates cap-independent translation in a transcript-specific manner	Anees et al. (2021); Beijer et al. (2021); Bonnal et al. (2005); Cammas et al. (2007); Feng et al. (2022); Gui et al. (2019); Guil et al. (2006); Roy et al. (2014)
FMR/FXRs	+	+	Dimer	UBAP2/2L	−	No	Fragile X syndrome, fragile X tremor/ataxia syndrome, premature ovarian failure	Inhibits translation initiation and elongation in context-dependent manners	Dolen et al. (2007); Guo et al. (2014); Hagerman et al. (2017); Laggerbauer et al. (2001); Li et al. (2001); Sanders et al. (2020); Sopova et al. (2019); Sullivan et al. (2011)
Caprin	+	−	Dimer	G3BP1/2	−	No	Neurodevelopmental disease	Inhibits translation Promotes translation of synaptic proteins through RNA transport	Pavinato et al. (2022); Sanders et al. (2020); Shiina et al. (2010); Solomon et al. (2007)
TDP-43	+	+	Di/Oligomer	ATXN2, hnRNP A/Bs	−/+	n/a	ALS, ALS-FTD, frontotemporal lobar degeneration	Promotes translation of specific mRNAs, and at axons through RNA transport Inhibits translation by sequestration in RNP granules	Altman et al. (2021); Briese et al. (2020); Buratti et al. (2005); Nagano et al. (2020); Nonaka and Hasegawa (2018); Sreedharan et al. (2008)
FUS	+	+	Oligomer	n/a	n/a	n/a	ALS, ALS-FTD, hereditary essential tremor	Promotes local translation within RNP granules Cytoplasmic FUS mutants are associated with translation suppression	Kamelgarn et al. (2018); Lopez-Erauskin et al. (2018); Shorter (2017); Yasuda et al. (2013)
ATXN2/L	+	−	Dimer	TDP-43, G3BP1	−/+	Yes	Spinocerebellar ataxia type 2, ALS, late-onset Parkinson's disease	Promotes translation of specific mRNAs, ablation decreases global translation rate	Baumer et al. (2014); Elden et al. (2010); Fittschen et al. (2015); Kaehler et al. (2012); Li et al. (2022); Yamashita et al. (2014)

The presence of IDR or LCD domains, prion-like behavior, multimerization behavior, and demonstrated interactions with other SG RNA binding proteins is listed for key RBPs found in SGs. Also shown is the impact of genetic disruption or overexpression on SG formation, associated genetic diseases, and identified roles in translation for each. Proteins with IDR/LCD or prion-like domains are indicated with “+,” while those proteins that do not have these regions are indicated with “−.” SG formation is: ++: prevented, +: greatly decreased; −/+: decreased in some studies; or −: unaffected by genetic disruption of the indicated proteins.

ALS, amyotrophic lateral sclerosis; FTD, frontotemporal dementia; IDR, intrinsically disordered region; LCD, low-complexity domain; mRNA, messenger RNA; n/a, not available; RBP, RNA-binding protein; RNP, ribonucleoprotein; TDP-43, TAR DNA binding protein; TIA-1, TIA1 cytotoxic granule associated RNA binding protein; TIAR, TIA1 cytotoxic granule associated RNA binding protein like 1; n/a, data not available.