Table 1.

Human RNA-binding proteins with prion-like domains

Protein RRM domain (Pfam PF00076) RNA binding (GO:0003723) Prion domain score Prion domain rank P-bodies and/or SGs? Neurodegenerative disease? 
TNRC6A − 42.1  
FUS 38.5 12 ALS, FTLD 
ATXN1 − 35.5 15  SCA1 
TAF15 33.2 22 ALS, FTLD 
EWSR1 32.3 25 ALS, FTLD 
HNRPDL 31.5 27   
HNRNPD 30.7 29.5   
HNRNPA2B1 29.9 32 IBMPFD 
ILF3 − 29.8 33   
HNRNPUL1 − 28.5 37   
HNRNPA1 28.2 38 IBMPFD, ALS 
HNRNPAB 27.8 39   
HNRNPA3 27.2 41 C9orf72 ALS/FTLD 
TARDBP 26.5 43 ALS, FTLD 
HNRNPU − 24.9 49   
TIA1 23.4 53 Welander distal myopathy 
HNRNPA1L2 22.8 57   
HNRNPH1 22.2 63   
DDX5 − 21.2 73  
PSF 20.8 79  FTLD 
HNRNPA0 20.5 81   
HNRNPH2 17.5 101   
DAZ2 16.2 119   
RBM14 16.0 122   
CSTF2 15.7 126   
TNRC6C − 15.4 128  
SOX2 − 15.0 135   
CAPRIN1 − 14.9 136  
DROSHA − 14.8 137   
DDX17 − 14.8 139   
DAZ3 14.6 144.5   
DAZ2 14.6 144.5   
DAZ1 14.1 148   
HNRNPH3 14.0 151   
CSTF2T 14.0 153   
CELF4 13.8 156   
TIAL1 13.6 162  
RBM33 12.9 178   
DLX2 − 12.5 188   
DAZAP1 11.7 203   
SUPT6H − 11.6 206   
ATXN2 − 10.2 227 SCA2, ALS 
DHX9 − 10.1 230   
PSPC1 10.0 231   
GAR1 − 9.4 235   
SF1 − 9.4 236   
FUBP1 − 9.2 237   
EIF4G3 − 8.5 243  
EIF4G1 − 8.3 246 PD 
Protein RRM domain (Pfam PF00076) RNA binding (GO:0003723) Prion domain score Prion domain rank P-bodies and/or SGs? Neurodegenerative disease? 
TNRC6A − 42.1  
FUS 38.5 12 ALS, FTLD 
ATXN1 − 35.5 15  SCA1 
TAF15 33.2 22 ALS, FTLD 
EWSR1 32.3 25 ALS, FTLD 
HNRPDL 31.5 27   
HNRNPD 30.7 29.5   
HNRNPA2B1 29.9 32 IBMPFD 
ILF3 − 29.8 33   
HNRNPUL1 − 28.5 37   
HNRNPA1 28.2 38 IBMPFD, ALS 
HNRNPAB 27.8 39   
HNRNPA3 27.2 41 C9orf72 ALS/FTLD 
TARDBP 26.5 43 ALS, FTLD 
HNRNPU − 24.9 49   
TIA1 23.4 53 Welander distal myopathy 
HNRNPA1L2 22.8 57   
HNRNPH1 22.2 63   
DDX5 − 21.2 73  
PSF 20.8 79  FTLD 
HNRNPA0 20.5 81   
HNRNPH2 17.5 101   
DAZ2 16.2 119   
RBM14 16.0 122   
CSTF2 15.7 126   
TNRC6C − 15.4 128  
SOX2 − 15.0 135   
CAPRIN1 − 14.9 136  
DROSHA − 14.8 137   
DDX17 − 14.8 139   
DAZ3 14.6 144.5   
DAZ2 14.6 144.5   
DAZ1 14.1 148   
HNRNPH3 14.0 151   
CSTF2T 14.0 153   
CELF4 13.8 156   
TIAL1 13.6 162  
RBM33 12.9 178   
DLX2 − 12.5 188   
DAZAP1 11.7 203   
SUPT6H − 11.6 206   
ATXN2 − 10.2 227 SCA2, ALS 
DHX9 − 10.1 230   
PSPC1 10.0 231   
GAR1 − 9.4 235   
SF1 − 9.4 236   
FUBP1 − 9.2 237   
EIF4G3 − 8.5 243  
EIF4G1 − 8.3 246 PD 

49 human RNA-binding proteins (containing the Pfam RRM [PF00076] and/or annotated with the GO term for RNA-binding [GO:0003723]) also harbor predicted prion-like domains. Prion score, based on Alberti et al. (2009), indicates the maximum log-likelihood ratio for prion-like amino acid composition vs. non–prion-like amino acid composition in any 60 consecutive amino acid window contained in a region parsed as prion-like by the hidden Markov model. Prion domain rank is from 21,873 human proteins, 250 of which had positive prion score. Localization to P-bodies or SGs is based on literature searches, especially Buchan and Parker (2009). Human disease connections for select proteins are indicated.

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