Table 1.
Membrane-less organelles and the corresponding RNA-binding proteins.
| Location | RNA-Binding Proteins | Function |
|---|---|---|
| Stress granules | FUS | Abnormal stress granule formation [32,93] |
| TARDBP | Abnormal stress granule formation [33,85]; GR or PR DPR interactor [47,96] | |
| ATXN2 | Stress granule components; cause ALS or increase its risk [16,25] | |
| TIA-1 | Stress granule components; abnormal stress granule dynamics [28,91]; GR or PR DPR interactor [47] | |
| HNRNPA2B1 | Formation of aberrant stress granules [26]; GR or PR DPR interactor [47,101] | |
| HNRNPA1 | Increased incorporation into stress granules [94]; GR or PR DPR interactor [47,102] | |
| G3BP1 | GR or PR DPR interactor [47,99] | |
| PABPC1 | GR or PR DPR interactor [47,101] | |
| PTBP1 | GR or PR DPR interactor [47,101] | |
| SFPQ | GR or PR DPR interactor [47] | |
| YBX1 | GR or PR DPR interactor [47,101] | |
| HNRNPUL1 | GR or PR DPR interactor [47] | |
| FXR | GR or PR DPR interactor [47] | |
| LBR | GR or PR DPR interactor [47] | |
| SYNCRIP | GR or PR DPR interactor [47,101] | |
| HNRNPH | GR or PR DPR interactor [47,101] | |
| PFN1 | Impairments in stress granule dynamics [80,104] | |
| SOD1 | Incorporation into stress granule; delaying stress granule assembly [105,106] | |
| Nucleolus | NPM1 | Colocalization and interaction with GR or PR DPR [47,109]; mislocalization with impaired nucleolus dynamics [118,120] |
| NCL | Colocalization and interaction with GR or PR DPR also with repeat RNA [38,47,118,119], altered distribution, and nucleolar stress [122,123] | |
| FBL | Colocalization DPR [109,155] | |
| ADARB2 | Interaction with repeat RNA of C9orf72 [38] | |
| HNRNPA3 | Interaction with repeat RNA of C9orf72 [38] | |
| HNRNPH | Interaction with repeat RNA of C9orf72 [38] | |
| Cajal bodies | SRRM2 | GR and PR DPR interactor [47]; DPR altered dynamics [69] |
| FUS | Decreasing number of Cajal bodies [69]; altering distribution of snRNP into Cajal bodies [68,69] | |
| Nuclear speckles | SRSF7 | Altered dynamics by interaction with GR or PR DPR [47] |
| SRSF2 | Colocalize with C9orf72 RNA foci [38,133] | |
| TARDBP | Disruption of nuclear speckles [127,135]; disturbing splicing event [136] and expression of MALAT1 [137] | |
| FUS | Disruption of nuclear speckles [127] | |
| Paraspeckles | SFPQ | Paraspeckle-enriched and mutation in ALS [141,144], associated with C9orf72 [138,150,151] |
| FUS | Paraspeckle-enriched and mutation in ALS [141,143,145], dysfunction of paraspeckle [149], associated with C9orf72 RNA foci [150] | |
| EWSR1 | Paraspeckle-enriched and mutation in ALS [24,143] | |
| TAF15 | Paraspeckle-enriched and mutation in ALS [141,143] | |
| TARDBP | Paraspeckle-enriched and mutation in ALS [141,143,145]; defect in paraspeckle [146,147] | |
| SS18L1 | Paraspeckle-enriched and mutation in ALS [141,143] | |
| HNRNPA1 | Paraspeckle-enriched and mutation in ALS [141,143] | |
| NONO | Associated with C9orf72 RNA foci [150] and DPR [138,151] | |
| RBM14 | Associated with C9orf72 RNA foci [150] and DPR [138,151] | |
| HNRNPH | Associated with C9orf72 RNA foci [150] and regulated by poly PR [138] | |
| HNRNPF | GR or PR DPR interactor [138,151] and regulated by poly PR [138] | |
| HNRNPM | GR or PR DPR interactor [138,151] | |
| FAM98 | GR or PR DPR interactor [138,151] | |
| MATR3 | GR or PR DPR interactor [138,151] | |
| Nucleocytoplasmic shuttling | FUS | Dysregulation of nucleocytoplasmic shuttling [32,63] |
| TARDBP | Dysregulation of nucleocytoplasmic shuttling [33,61] | |
| HNRNPA3 | Nucleocytoplasmic transport capability [71]; mislocalization by DPR [75] | |
| MATR3 | Sequestrated by RNA foci [73] | |
| PARP1 | GR or PR DPR interactor; nucleocytoplasmic shuttling modulator [47] | |
| YBX1 | GR or PR DPR interactor; nucleocytoplasmic shuttling modulator [47] | |
| LBR | GR or PR DPR interactor; nucleocytoplasmic shuttling modulator [47] | |
| U2 snRNP | Mislocalization by DPR [74] | |
| HNRNPA1 | Cytoplasmic inclusion formation [26] | |
| HNRNPA2B1 | Cytoplasmic inclusion formation [26] | |
| PFN1 | Altered distribution of RNA-binding proteins [80] |