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 | 9 | + | |
| 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 | 9 | + | |
| 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.