Table 1.

Summary of RNA-binding proteins associated with RNA granules and linked to neurological diseases

RNA-binding protein	RNA granule	Link to disease	References
Angiogenin (ANG)	SG	Mutations in ALS and PD	[37, 98, 99]
Ataxin-2 (ATXN2)	SG	PolyQ expansions in ALS and SCA2	[27, 36, 95, 96]
Ewing sarcoma protein (EWS)	SG	Mutations in ALS, inclusions in FTD	[38, 100, 101]
Fragile X mental retardation protein (FMRP)	tRNP, SG, PB	Mutations in FXS	[19, 27, 44, 59]
Fused in sarcoma (FUS)	tRNP, SG	Mutations and inclusions in ALS, FTD & PQE	[6, 24, 35, 38, 104]
Heterogeneous nuclear ribonuclearprotein (hnRNPA2B1)	SG	Mutations in ALS, FTD and PGD	[39]
Heterogeneous nuclear ribonuclearprotein (hnRNPA1)	SG	Mutations in ALS and PGD	[39]
Survival of motor neuron (SMN)	tRNP, SG	Mutations in ALS and SMA	[19, 27, 40, 92–94]
TATA-binding protein-associated factor 15 (TAF15)	tRNP, SG, PB	Mutations in ALS, inclusions in ALS and FTD	[25, 38, 100, 102, 103]
TAR DNA-binding protein (TDP-43)	tRNP, SG, PB	Mutations in ALS, FTD, inclusions in AD and HD	[7, 22, 29, 105, 106]

AD Alzheimer’s disease, ALS amyotrophic lateral sclerosis, FTD frontotemporal dementia, FXS fragile X syndrome, HD Huntington’s disease, PB processing body, PD Parkinson’s disease, PGD Paget disease, PQE polyQ expansion disease, tRNP transport ribonucleoprotein particle granule, SCA2 spinocerebellar ataxia type 2, SG stress granule, SMA spinal muscular atrophy