{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T18:40:08Z","timestamp":1791830408087,"version":"4.3.4"},"reference-count":185,"publisher":"Springer Science and Business Media LLC","issue":"6","license":[{"start":{"date-parts":[[2023,9,30]],"date-time":"2023-09-30T00:00:00Z","timestamp":1696032000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2023,9,30]],"date-time":"2023-09-30T00:00:00Z","timestamp":1696032000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Neurotox Res"],"published-print":{"date-parts":[[2023,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Despite sustained efforts to treat neurodegenerative diseases, little is known at the molecular level to understand and generate novel therapeutic approaches for these malignancies. Therefore, it is not surprising that neurogenerative diseases are among the leading causes of death in the aged population. Neurons require sophisticated cellular mechanisms to maintain proper protein homeostasis. These cells are generally sensitive to loss of gene expression control at the post-transcriptional level. Post-translational control responds to signals that can arise from intracellular processes or environmental factors that can be regulated through RNA-binding proteins. These proteins recognize RNA through one or more RNA-binding domains and form ribonucleoproteins that are critically involved in the regulation of post-transcriptional processes from splicing to the regulation of association of the translation machinery allowing a relatively rapid and precise modulation of the transcriptome. Neurotoxicity is the result of the biological, chemical, or physical interaction of agents with an adverse effect on the structure and function of the central nervous system. The disruption of the proper levels or function of RBPs in neurons and glial cells triggers neurotoxic events that are linked to neurodegenerative diseases such as spinal muscular atrophy (SMA), amyotrophic lateral sclerosis (ALS), fragile X syndrome (FXS), and frontotemporal dementia (FTD) among many others. The connection between RBPs and neurodegenerative diseases opens a new landscape for potentially novel therapeutic targets for the intervention of these neurodegenerative pathologies. In this contribution, a summary of the recent findings of the molecular mechanisms involved in the plausible role of RBPs in RNA processing in neurodegenerative disease is discussed.<\/jats:p>","DOI":"10.1007\/s12640-023-00669-w","type":"journal-article","created":{"date-parts":[[2023,9,30]],"date-time":"2023-09-30T12:01:41Z","timestamp":1696075301000},"page":"681-697","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["RNA-Binding Proteins: A Role in Neurotoxicity?"],"prefix":"10.1007","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1986-6299","authenticated-orcid":false,"given":"Andrea","family":"Ochar\u00e1n-Mercado","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3362-7136","authenticated-orcid":false,"given":"Jaqueline","family":"Loaeza-Loaeza","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9533-6241","authenticated-orcid":false,"given":"Yaneth","family":"Castro-Coronel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0067-0488","authenticated-orcid":false,"given":"Leonor C.","family":"Acosta-Saavedra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2710-3731","authenticated-orcid":false,"given":"Luisa C.","family":"Hern\u00e1ndez-Kelly","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6201-5553","authenticated-orcid":false,"given":"Daniel","family":"Hern\u00e1ndez-Sotelo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9594-8114","authenticated-orcid":false,"given":"Arturo","family":"Ortega","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,9,30]]},"reference":[{"key":"669_CR1","doi-asserted-by":"publisher","unstructured":"Adeli\u00a0K (2011) Translational control mechanisms in metabolic regulation: critical role of RNA binding proteins, microRNAs, and cytoplasmic RNA granules. Am J Physiol Endocrinol Metabol 301 (6).\u00a0https:\/\/doi.org\/10.1152\/ajpendo.00399.2011","DOI":"10.1152\/ajpendo.00399.2011"},{"issue":"1","key":"669_CR2","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1016\/bs.mie.2015.01.015","volume":"558","author":"T Afroz","year":"2015","unstructured":"Afroz T, Cienikova Z, Cl\u00e9ry A, Allain FHT (2015) One, two, three, four! How multiple RRMs read the genome sequence. Methods Enzymol 558(1):235\u2013278. https:\/\/doi.org\/10.1016\/bs.mie.2015.01.015","journal-title":"Methods Enzymol"},{"key":"669_CR3","doi-asserted-by":"crossref","unstructured":"Ainsley JA, Drane L, Jacobs J, Kittelberger KA, Reijmers LG (2014) Functionally diverse dendritic mRNAs rapidly associate with ribosomes following a novel experience,\u201d (in eng). Nat Commun\u00a05:4510","DOI":"10.1038\/ncomms5510"},{"issue":"1","key":"669_CR4","doi-asserted-by":"publisher","first-page":"78","DOI":"10.4331\/wjbc.v7.i1.78","volume":"7","author":"LR Alves","year":"2016","unstructured":"Alves LR (2016) RNA-binding proteins related to stress response and differentiation in protozoa. World J Biol Chem 7(1):78\u201378. https:\/\/doi.org\/10.4331\/wjbc.v7.i1.78","journal-title":"World J Biol Chem"},{"issue":"6","key":"669_CR5","doi-asserted-by":"publisher","first-page":"430","DOI":"10.1038\/nrm2694","volume":"10","author":"P Anderson","year":"2009","unstructured":"Anderson P, Kedersha N (2009) RNA granules: post-transcriptional and epigenetic modulators of gene expression. Nat Rev Mol Cell Biol 10(6):430\u2013436. https:\/\/doi.org\/10.1038\/nrm2694","journal-title":"Nat Rev Mol Cell Biol"},{"issue":"7","key":"669_CR6","doi-asserted-by":"publisher","first-page":"861","DOI":"10.1016\/j.bbagrm.2014.11.009","volume":"1849","author":"P Anderson","year":"2015","unstructured":"Anderson P, Kedersha N, Ivanov P (2015) Stress granules, P-bodies and cancer. Biochimica Et Biophysica Acta - Gene Regulatory Mechanisms 1849(7):861\u2013870. https:\/\/doi.org\/10.1016\/j.bbagrm.2014.11.009","journal-title":"Biochimica Et Biophysica Acta - Gene Regulatory Mechanisms"},{"issue":"1","key":"669_CR7","doi-asserted-by":"publisher","first-page":"72","DOI":"10.1038\/s41593-017-0022-z","volume":"21","author":"DJ Apicco","year":"2018","unstructured":"Apicco DJ et al (2018) Reducing the RNA binding protein TIA1 protects against tau-mediated neurodegeneration in vivo. Nat Neurosci 21(1):72\u201382. https:\/\/doi.org\/10.1038\/s41593-017-0022-z","journal-title":"Nat Neurosci"},{"issue":"1","key":"669_CR8","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1111\/j.1365-2990.2011.01188.x","volume":"38","author":"RA Armstrong","year":"2012","unstructured":"Armstrong RA, Carter D, Cairns NJ (2012) A quantitative study of the neuropathology of 32 sporadic and familial cases of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP). Neuropathol Appl Neurobiol 38(1):25\u201338. https:\/\/doi.org\/10.1111\/j.1365-2990.2011.01188.x","journal-title":"Neuropathol Appl Neurobiol"},{"issue":"16","key":"669_CR9","doi-asserted-by":"publisher","first-page":"3206","DOI":"10.1093\/hmg\/ddq230","volume":"19","author":"PEA Ash","year":"2010","unstructured":"Ash PEA et al (2010) Neurotoxic effects of TDP-43 overexpression in C. elegans. Hum Mol Genet 19(16):3206\u20133218. https:\/\/doi.org\/10.1093\/hmg\/ddq230","journal-title":"Hum Mol Genet"},{"issue":"1","key":"669_CR10","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1186\/gb4153","volume":"15","author":"N Attar","year":"2014","unstructured":"Attar N (2014) The RBPome: Where the brains meet the brawn. Genome Biol 15(1):1\u20135. https:\/\/doi.org\/10.1186\/gb4153","journal-title":"Genome Biol"},{"issue":"5","key":"669_CR11","doi-asserted-by":"publisher","first-page":"599","DOI":"10.1007\/s00401-020-02203-0","volume":"140","author":"A Bampton","year":"2020","unstructured":"Bampton A, Gittings LM, Fratta P, Lashley T, Gatt A (2020) The role of hnRNPs in frontotemporal dementia and amyotrophic lateral sclerosis. Acta Neuropathol 140(5):599\u2013623. https:\/\/doi.org\/10.1007\/s00401-020-02203-0","journal-title":"Acta Neuropathol"},{"issue":"17","key":"669_CR12","doi-asserted-by":"publisher","first-page":"4230","DOI":"10.1111\/febs.12294","volume":"280","author":"A Banerjee","year":"2013","unstructured":"Banerjee A, Apponi LH, Pavlath GK, Corbett AH (2013) PABPN1: Molecular function and muscle disease. FEBS J 280(17):4230\u20134250. https:\/\/doi.org\/10.1111\/febs.12294","journal-title":"FEBS J"},{"key":"669_CR13","doi-asserted-by":"publisher","first-page":"35","DOI":"10.1007\/82_2011_145","volume":"353","author":"P Barraud","year":"2012","unstructured":"Barraud P, Allain FH (2012) \u201cADAR proteins: double-stranded RNA and Z-DNA binding domains\u201d, (in eng). Curr Top Microbiol Immunol 353:35\u201360. https:\/\/doi.org\/10.1007\/82_2011_145","journal-title":"Curr Top Microbiol Immunol"},{"key":"669_CR14","doi-asserted-by":"crossref","unstructured":"Bartel DP, Xiang\u00a0K (2021) The molecular basis of coupling between poly(A)-tail length and translational efficiency. bioRxiv 2021.01.18.427055","DOI":"10.1101\/2021.01.18.427055"},{"issue":"2","key":"669_CR15","doi-asserted-by":"publisher","first-page":"311","DOI":"10.1016\/j.molcel.2014.08.027","volume":"56","author":"R Batra","year":"2014","unstructured":"Batra R et al (2014) Loss of MBNL leads to disruption of developmentally regulated alternative polyadenylation in RNA-mediated disease. Mol Cell 56(2):311\u2013322. https:\/\/doi.org\/10.1016\/j.molcel.2014.08.027","journal-title":"Mol Cell"},{"issue":"6","key":"669_CR16","doi-asserted-by":"publisher","first-page":"1029","DOI":"10.1007\/s00424-016-1819-4","volume":"468","author":"BM Beckmann","year":"2016","unstructured":"Beckmann BM, Castello A, Medenbach J (2016) The expanding universe of ribonucleoproteins: of novel RNA-binding proteins and unconventional interactions. Pflugers Arch 468(6):1029\u20131040. https:\/\/doi.org\/10.1007\/s00424-016-1819-4","journal-title":"Pflugers Arch"},{"key":"669_CR17","doi-asserted-by":"publisher","unstructured":"Biswas J, Nunes L, Das S, Yoon YJ, Eliscovich C, Singer RH (2019) Zipcode binding protein 1 (ZBP1; IGF2BP1): a model for sequence specific RNA regulation. Cold Spring Harb Symp Quant Biol\u00a084:1\u201310. https:\/\/doi.org\/10.1101\/sqb.2019.84.039396.Zipcode","DOI":"10.1101\/sqb.2019.84.039396.Zipcode"},{"issue":"1","key":"669_CR18","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1371\/journal.pgen.1005827","volume":"12","author":"E Bondy-Chorney","year":"2016","unstructured":"Bondy-Chorney E et al (2016) Staufen1 regulates multiple alternative splicing events either positively or negatively in DM1 indicating its role as a disease modifier. PLoS Genet 12(1):1\u201322. https:\/\/doi.org\/10.1371\/journal.pgen.1005827","journal-title":"PLoS Genet"},{"issue":"3","key":"669_CR19","doi-asserted-by":"publisher","first-page":"400","DOI":"10.1038\/s12276-020-0407-z","volume":"52","author":"SH Boo","year":"2020","unstructured":"Boo SH, Kim YK (2020) The emerging role of RNA modifications in the regulation of mRNA stability. Exp Mol Med 52(3):400\u2013408. https:\/\/doi.org\/10.1038\/s12276-020-0407-z","journal-title":"Exp Mol Med"},{"issue":"4","key":"669_CR20","doi-asserted-by":"publisher","first-page":"856","DOI":"10.1042\/bst20120100","volume":"40","author":"M Brook","year":"2012","unstructured":"Brook M, Gray NK (2012) The role of mammalian poly(A)-binding proteins in co-ordinating mRNA turnover. Biochem Soc Trans 40(4):856\u2013864. https:\/\/doi.org\/10.1042\/bst20120100","journal-title":"Biochem Soc Trans"},{"issue":"1","key":"669_CR21","doi-asserted-by":"publisher","first-page":"169","DOI":"10.1111\/gbb.12273","volume":"15","author":"CD Bryant","year":"2016","unstructured":"Bryant CD, Yazdani N (2016) RNA-binding proteins, neural development and the addictions. Genes Brain Behav 15(1):169\u2013186. https:\/\/doi.org\/10.1111\/gbb.12273","journal-title":"Genes Brain Behav"},{"issue":"6","key":"669_CR22","doi-asserted-by":"publisher","first-page":"932","DOI":"10.1016\/j.molcel.2009.11.020","volume":"36","author":"JR Buchan","year":"2009","unstructured":"Buchan JR, Parker R (2009) Eukaryotic stress granules\u202f: the ins and out of translation what are stress granules\u202f? Mol Cell 36(6):932\u2013932","journal-title":"Mol Cell"},{"issue":"4","key":"669_CR23","doi-asserted-by":"publisher","first-page":"657","DOI":"10.1016\/0896-6273(93)90077-5","volume":"11","author":"RJ Buckanovich","year":"1993","unstructured":"Buckanovich RJ, Posner JB, Darnell RB (1993) Nova, the paraneoplastic Ri antigen, is homologous to an RNA-binding protein and is specifically expressed in the developing motor system. Neuron 11(4):657\u2013672. https:\/\/doi.org\/10.1016\/0896-6273(93)90077-5","journal-title":"Neuron"},{"issue":"5172","key":"669_CR24","doi-asserted-by":"publisher","first-page":"615","DOI":"10.1126\/science.8036511","volume":"265","author":"CG Burd","year":"1994","unstructured":"Burd CG, Dreyfusst G (1994) Conserved structures. Science 265(5172):615\u2013621","journal-title":"Science"},{"issue":"11","key":"669_CR25","doi-asserted-by":"publisher","first-page":"662","DOI":"10.1016\/j.tibs.2015.08.012","volume":"40","author":"S Calabretta","year":"2015","unstructured":"Calabretta S, Richard S (2015) Emerging Roles of Disordered Sequences in RNA-Binding Proteins. Trends Biochem Sci 40(11):662\u2013672. https:\/\/doi.org\/10.1016\/j.tibs.2015.08.012","journal-title":"Trends Biochem Sci"},{"issue":"4","key":"669_CR26","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1111\/acel.13136","volume":"19","author":"X Cao","year":"2020","unstructured":"Cao X, Jin X, Liu B (2020) The involvement of stress granules in aging and aging-associated diseases. Aging Cell 19(4):1\u201320. https:\/\/doi.org\/10.1111\/acel.13136","journal-title":"Aging Cell"},{"key":"669_CR27","doi-asserted-by":"publisher","unstructured":"Celona B et al (2017) Suppression of C9orf72 RNA repeat-induced neurotoxicity by the ALS-associated RNA-binding protein Zfp106. Elife 6. https:\/\/doi.org\/10.7554\/eLife.19032","DOI":"10.7554\/eLife.19032"},{"issue":"9","key":"669_CR28","doi-asserted-by":"publisher","first-page":"2109","DOI":"10.1111\/j.1742-4658.2005.04652.x","volume":"272","author":"KY Chang","year":"2005","unstructured":"Chang KY, Ramos A (2005) The double-stranded RNA-binding motif, a versatile macromolecular docking platform. FEBS J 272(9):2109\u20132117. https:\/\/doi.org\/10.1111\/j.1742-4658.2005.04652.x","journal-title":"FEBS J"},{"issue":"1","key":"669_CR29","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1186\/s13578-021-00581-w","volume":"11","author":"Y Chao","year":"2021","unstructured":"Chao Y et al (2021) Regulatory roles and mechanisms of alternative RNA splicing in adipogenesis and human metabolic health. Cell Biosci 11(1):1\u201316. https:\/\/doi.org\/10.1186\/s13578-021-00581-w","journal-title":"Cell Biosci"},{"issue":"9","key":"669_CR30","doi-asserted-by":"publisher","first-page":"2088","DOI":"10.1111\/j.1742-4658.2005.04650.x","volume":"272","author":"Y Chen","year":"2005","unstructured":"Chen Y, Varani G (2005) Protein families and RNA recognition. FEBS J 272(9):2088\u20132097. https:\/\/doi.org\/10.1111\/j.1742-4658.2005.04650.x","journal-title":"FEBS J"},{"issue":"1\u20132","key":"669_CR31","doi-asserted-by":"publisher","first-page":"56","DOI":"10.1016\/j.cell.2018.11.030","volume":"176","author":"JM Cioni","year":"2019","unstructured":"Cioni JM et al (2019) Late endosomes act as mRNA translation platforms and sustain mitochondria in axons. Cell 176(1\u20132):56-72.e15. https:\/\/doi.org\/10.1016\/j.cell.2018.11.030","journal-title":"Cell"},{"issue":"8","key":"669_CR32","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1101\/cshperspect.a012344","volume":"4","author":"JC Darnell","year":"2012","unstructured":"Darnell JC, Richter JD (2012) Cytoplasmic RNA-binding proteins and the control of complex brain function. Cold Spring Harb Perspect Biol 4(8):1\u201317. https:\/\/doi.org\/10.1101\/cshperspect.a012344","journal-title":"Cold Spring Harb Perspect Biol"},{"key":"669_CR33","doi-asserted-by":"publisher","unstructured":"Das S, Singer RH, Yoon\u00a0Y (2019) The travels of mRNAs in neurons: do they know where they are going?. Physiol Behav\u00a057:110\u2013116. https:\/\/doi.org\/10.1016\/j.conb.2019.01.016.The","DOI":"10.1016\/j.conb.2019.01.016.The"},{"issue":"2","key":"669_CR34","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1002\/wrna.1394","volume":"8","author":"L De Conti","year":"2017","unstructured":"De Conti L, Baralle M, Buratti E (2017) Neurodegeneration and RNA-binding proteins. Wiley Interdisciplinary Reviews: RNA 8(2):1\u201312. https:\/\/doi.org\/10.1002\/wrna.1394","journal-title":"Wiley Interdisciplinary Reviews: RNA"},{"issue":"9","key":"669_CR35","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1101\/cshperspect.a012286","volume":"4","author":"CJ Decker","year":"2012","unstructured":"Decker CJ, Parker R (2012) P-bodies and stress granules: possible roles in the control of translation and mRNA degradation. Cold Spring Harb Perspect Biol 4(9):1\u201316","journal-title":"Cold Spring Harb Perspect Biol"},{"issue":"5","key":"669_CR36","doi-asserted-by":"publisher","first-page":"1320","DOI":"10.1093\/brain\/awy062","volume":"141","author":"JE Deshaies","year":"2018","unstructured":"Deshaies JE et al (2018) TDP-43 regulates the alternative splicing of hnRNP A1 to yield an aggregation-prone variant in amyotrophic lateral sclerosis. Brain 141(5):1320\u20131333. https:\/\/doi.org\/10.1093\/brain\/awy062","journal-title":"Brain"},{"issue":"6","key":"669_CR37","doi-asserted-by":"publisher","first-page":"926","DOI":"10.1016\/j.devcel.2008.04.003","volume":"14","author":"JB Dictenberg","year":"2008","unstructured":"Dictenberg JB, Swanger SA, Antar LN, Singer RH, Bassell GJ (2008) A direct role for FMRP in activity-dependent dendritic mRNA transport links filopodial-spine morphogenesis to fragile X syndrome. Dev Cell 14(6):926\u2013939. https:\/\/doi.org\/10.1016\/j.devcel.2008.04.003","journal-title":"Dev Cell"},{"issue":"3","key":"669_CR38","doi-asserted-by":"publisher","first-page":"233","DOI":"10.1038\/s41422-019-0141-z","volume":"29","author":"Y Duan","year":"2019","unstructured":"Duan Y et al (2019) PARylation regulates stress granule dynamics, phase separation, and neurotoxicity of disease-related RNA-binding proteins. Cell Res 29(3):233\u2013247. https:\/\/doi.org\/10.1038\/s41422-019-0141-z","journal-title":"Cell Res"},{"key":"669_CR39","doi-asserted-by":"publisher","unstructured":"Duszczyk\u00a0M et al\u00a0(2019) The solution structure of Dead End bound to AU-rich RNA reveals an unprecedented mode of tandem RRM-RNA recognition required for mRNA regulation. BioRxiv 572156\u2013572156. https:\/\/doi.org\/10.1101\/572156","DOI":"10.1101\/572156"},{"issue":"2","key":"669_CR40","doi-asserted-by":"publisher","first-page":"364","DOI":"10.1016\/j.celrep.2018.12.045","volume":"26","author":"SY Ebstein","year":"2019","unstructured":"Ebstein SY, Yagudayeva I, Shneider NA (2019) Mutant TDP-43 causes early-stage dose-dependent motor neuron degeneration in a TARDBP knockin mouse model of ALS. Cell Rep 26(2):364-373.e4. https:\/\/doi.org\/10.1016\/j.celrep.2018.12.045","journal-title":"Cell Rep"},{"key":"669_CR41","doi-asserted-by":"publisher","first-page":"38","DOI":"10.1016\/j.ceb.2017.02.008","volume":"45","author":"C Eliscovich","year":"2017","unstructured":"Eliscovich C, Singer RH (2017) RNP transport in cell biology: the long and winding road. Curr Opin Cell Biol 45:38\u201346. https:\/\/doi.org\/10.1016\/j.ceb.2017.02.008","journal-title":"Curr Opin Cell Biol"},{"issue":"1","key":"669_CR42","doi-asserted-by":"publisher","first-page":"96","DOI":"10.1261\/rna.037044.112","volume":"19","author":"S Erkelenz","year":"2013","unstructured":"Erkelenz S et al (2013) Position-dependent splicing activation and repression by SR and hnRNP proteins rely on common mechanisms. RNA 19(1):96\u2013102. https:\/\/doi.org\/10.1261\/rna.037044.112","journal-title":"RNA"},{"key":"669_CR43","first-page":"15293","volume":"95","author":"XC Fan","year":"1998","unstructured":"Fan XC, Steitz JA (1998) HNS, a nuclear-cytoplasmic shuttling sequence in HuR (nuclear localization\u035eRNA degradation\u035enuclear export). Biochemistry 95:15293\u201315298","journal-title":"Biochemistry"},{"issue":"12","key":"669_CR44","doi-asserted-by":"publisher","first-page":"3573","DOI":"10.1016\/j.celrep.2017.11.093","volume":"21","author":"MM Fay","year":"2017","unstructured":"Fay MM, Anderson PJ, Ivanov P (2017) ALS\/FTD-associated C9ORF72 repeat RNA promotes phase transitions in vitro and in cells. Cell Rep 21(12):3573\u20133584. https:\/\/doi.org\/10.1016\/j.celrep.2017.11.093","journal-title":"Cell Rep"},{"issue":"10","key":"669_CR45","doi-asserted-by":"publisher","first-page":"1586","DOI":"10.1016\/j.febslet.2009.04.019","volume":"583","author":"F Feiguin","year":"2009","unstructured":"Feiguin F, Godena VK, Romano G, D\u2019Ambrogio A, Klima R, Baralle FE (2009) Depletion of TDP-43 affects Drosophila motoneurons terminal synapsis and locomotive behavior. FEBS Lett 583(10):1586\u20131592. https:\/\/doi.org\/10.1016\/j.febslet.2009.04.019","journal-title":"FEBS Lett"},{"issue":"41","key":"669_CR46","doi-asserted-by":"publisher","first-page":"16999","DOI":"10.1074\/jbc.M117.797878","volume":"292","author":"N Filippova","year":"2017","unstructured":"Filippova N et al (2017) Hu antigen R (HuR) multimerization contributes to glioma disease progression. J Biol Chem 292(41):16999\u201317010. https:\/\/doi.org\/10.1074\/jbc.M117.797878","journal-title":"J Biol Chem"},{"issue":"2","key":"669_CR47","doi-asserted-by":"publisher","first-page":"893","DOI":"10.3390\/biom5020893","volume":"5","author":"AM Fredericks","year":"2015","unstructured":"Fredericks AM, Cygan KJ, Brown BA, Fairbrother WG (2015) RNA-binding proteins: Splicing factors and disease. Biomolecules 5(2):893\u2013909. https:\/\/doi.org\/10.3390\/biom5020893","journal-title":"Biomolecules"},{"issue":"17","key":"669_CR48","doi-asserted-by":"publisher","first-page":"2932","DOI":"10.1002\/1873-3468.13121","volume":"592","author":"C Gallagher","year":"2018","unstructured":"Gallagher C, Ramos A (2018) Joining the dots \u2013 protein-RNA interactions mediating local mRNA translation in neurons. FEBS Lett 592(17):2932\u20132947. https:\/\/doi.org\/10.1002\/1873-3468.13121","journal-title":"FEBS Lett"},{"key":"669_CR49","doi-asserted-by":"publisher","unstructured":"Garc\u00eda-Mauri\u00f1o\u00a0SM et al\u00a0(2017) RNA binding protein regulation and cross-talk in the control of AU-rich mRNA Fate. Front Mol Biosci 4:1\u20139. https:\/\/doi.org\/10.3389\/fmolb.2017.00071","DOI":"10.3389\/fmolb.2017.00071"},{"issue":"1","key":"669_CR50","doi-asserted-by":"publisher","first-page":"196","DOI":"10.1016\/j.molcel.2019.01.017","volume":"74","author":"M Garcia-Moreno","year":"2019","unstructured":"Garcia-Moreno M et al (2019) System-wide profiling of RNA-binding proteins uncovers key regulators of virus infection. Mol Cell 74(1):196-211.e11. https:\/\/doi.org\/10.1016\/j.molcel.2019.01.017","journal-title":"Mol Cell"},{"issue":"12","key":"669_CR51","doi-asserted-by":"publisher","first-page":"829","DOI":"10.1038\/nrg3813","volume":"15","author":"S Gerstberger","year":"2014","unstructured":"Gerstberger S, Hafner M, Tuschl T (2014) A census of human RNA-binding proteins. Nat Rev Genet 15(12):829\u2013845. https:\/\/doi.org\/10.1038\/nrg3813","journal-title":"Nat Rev Genet"},{"issue":"8","key":"669_CR52","doi-asserted-by":"publisher","first-page":"851","DOI":"10.1007\/s00439-016-1683-5","volume":"135","author":"T Geuens","year":"2016","unstructured":"Geuens T, Bouhy D, Timmerman V (2016) The hnRNP family: insights into their role in health and disease. Hum Genet 135(8):851\u2013867. https:\/\/doi.org\/10.1007\/s00439-016-1683-5","journal-title":"Hum Genet"},{"issue":"14","key":"669_CR53","doi-asserted-by":"publisher","first-page":"1977","DOI":"10.1016\/j.febslet.2008.03.004","volume":"582","author":"T Glisovic","year":"2008","unstructured":"Glisovic T, Bachorik JL, Yong J, Dreyfuss G (2008) RNA-binding proteins and post-transcriptional gene regulation. FEBS Lett 582(14):1977\u20131986. https:\/\/doi.org\/10.1016\/j.febslet.2008.03.004","journal-title":"FEBS Lett"},{"issue":"12","key":"669_CR54","doi-asserted-by":"publisher","first-page":"972","DOI":"10.1016\/j.tig.2018.09.006","volume":"34","author":"AC Goldstrohm","year":"2018","unstructured":"Goldstrohm AC, Hall TMT, McKenney KM (2018) Post-transcriptional regulatory functions of mammalian Pumilio proteins. Trends Genet 34(12):972\u2013990. https:\/\/doi.org\/10.1016\/j.tig.2018.09.006","journal-title":"Trends Genet"},{"issue":"2","key":"669_CR55","doi-asserted-by":"publisher","first-page":"149","DOI":"10.1136\/practneurol-2020-002730","volume":"21","author":"M Gowell","year":"2021","unstructured":"Gowell M, Baker I, Ansorge O, Husain M (2021) Young-onset frontotemporal dementia with FUS pathology. Pract Neurol 21(2):149\u2013152. https:\/\/doi.org\/10.1136\/practneurol-2020-002730","journal-title":"Pract Neurol"},{"key":"669_CR56","doi-asserted-by":"publisher","unstructured":"Grammatikakis I, Abdelmohsen K, Gorospe M (2017) Posttranslational control of HuR function. Wiley Interdisciplinary Reviews: RNA 8:(1). https:\/\/doi.org\/10.1002\/wrna.1372","DOI":"10.1002\/wrna.1372"},{"key":"669_CR57","doi-asserted-by":"publisher","unstructured":"Gregory JM, Fagegaltier D, Phatnani H, Harms MB\u00a0(2020) Genetics of amyotrophic lateral sclerosis. Curr Gen Med Rep 8(5):21\u2013131. https:\/\/doi.org\/10.1101\/cshperspect.a024125","DOI":"10.1101\/cshperspect.a024125"},{"issue":"32","key":"669_CR58","doi-asserted-by":"publisher","first-page":"8339","DOI":"10.1523\/jneurosci.0472-06.2006","volume":"26","author":"SY Grooms","year":"2006","unstructured":"Grooms SY et al (2006) Activity bidirectionally regulates AMPA receptor mRNA abundance in dendrites of hippocampal neurons. J Neurosci 26(32):8339\u20138351. https:\/\/doi.org\/10.1523\/jneurosci.0472-06.2006","journal-title":"J Neurosci"},{"issue":"3","key":"669_CR59","doi-asserted-by":"publisher","first-page":"253","DOI":"10.1007\/s40484-018-0148-3","volume":"6","author":"A Guvenek","year":"2018","unstructured":"Guvenek A, Tian B (2018) Analysis of alternative cleavage and polyadenylation in mature and differentiating neurons using RNA-seq data. Quant Biol 6(3):253\u2013266. https:\/\/doi.org\/10.1007\/s40484-018-0148-3","journal-title":"Quant Biol"},{"issue":"13","key":"669_CR60","doi-asserted-by":"publisher","first-page":"6561","DOI":"10.1021\/cr400514h","volume":"114","author":"J Habchi","year":"2014","unstructured":"Habchi J, Tompa P, Longhi S, Uversky VN (2014) Introducing protein intrinsic disorder. Chem Rev 114(13):6561\u20136588. https:\/\/doi.org\/10.1021\/cr400514h","journal-title":"Chem Rev"},{"issue":"2","key":"669_CR61","doi-asserted-by":"publisher","first-page":"685","DOI":"10.1128\/mcb.18.2.685","volume":"18","author":"LE Hake","year":"1998","unstructured":"Hake LE, Mendez R, Richter JD (1998) Specificity of RNA Binding by CPEB: Requirement for RNA Recognition Motifs and a Novel Zinc Finger. Mol Cell Biol 18(2):685\u2013693. https:\/\/doi.org\/10.1128\/mcb.18.2.685","journal-title":"Mol Cell Biol"},{"issue":"5","key":"669_CR62","doi-asserted-by":"publisher","first-page":"798","DOI":"10.1007\/s00018-007-7447-6","volume":"65","author":"RE Halbeisen","year":"2008","unstructured":"Halbeisen RE, Galgano A, Scherrer T, Gerber AP (2008) Post-transcriptional gene regulation: from genome-wide studies to principles. Cell Mol Life Sci 65(5):798\u2013813. https:\/\/doi.org\/10.1007\/s00018-007-7447-6","journal-title":"Cell Mol Life Sci"},{"key":"669_CR63","doi-asserted-by":"crossref","unstructured":"Hall DA, Berry-Kravis\u00a0E (2018) Fragile X syndrome and fragile X-associated tremor ataxia syndrome, 1 ed. (Handbook of Clinical Neurology). Elsevier BV 377\u2013391","DOI":"10.1016\/B978-0-444-63233-3.00025-7"},{"issue":"July","key":"669_CR64","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1038\/srep30805","volume":"6","author":"N Hamada","year":"2016","unstructured":"Hamada N et al (2016) Essential role of the nuclear isoform of RBFOX1, a candidate gene for autism spectrum disorders, in the brain development. Sci Rep 6(July):1\u201319. https:\/\/doi.org\/10.1038\/srep30805","journal-title":"Sci Rep"},{"issue":"SUPPL","key":"669_CR65","doi-asserted-by":"publisher","first-page":"3","DOI":"10.4103\/0973-1482.122506","volume":"9","author":"N Han","year":"2013","unstructured":"Han N, Li W, Zhang M (2013) The function of the RNA-binding protein hnRNP in cancer metastasis. J Cancer Res Ther 9(SUPPL):3. https:\/\/doi.org\/10.4103\/0973-1482.122506","journal-title":"J Cancer Res Ther"},{"issue":"15","key":"669_CR66","doi-asserted-by":"publisher","first-page":"11068","DOI":"10.1074\/jbc.C109.078527","volume":"285","author":"KA Hanson","year":"2010","unstructured":"Hanson KA, Kim SH, Wassarman DA, Tibbetts RS (2010) Ubiquilin modifies TDP-43 toxicity in a Drosophila model of amyotrophic lateral sclerosis (ALS). J Biol Chem 285(15):11068\u201311072. https:\/\/doi.org\/10.1074\/jbc.C109.078527","journal-title":"J Biol Chem"},{"issue":"1","key":"669_CR67","doi-asserted-by":"publisher","first-page":"46","DOI":"10.1002\/(sici)1097-4547(19981001)54:1<46::Aid-jnr6>3.0.Co;2-h","volume":"54","author":"RJ Hardy","year":"1998","unstructured":"Hardy RJ (1998) QKI expression is regulated during neuron-glial cell fate decisions. J Neurosci Res 54(1):46\u201357. https:\/\/doi.org\/10.1002\/(sici)1097-4547(19981001)54:1%3c46::Aid-jnr6%3e3.0.Co;2-h","journal-title":"J Neurosci Res"},{"issue":"18","key":"669_CR68","doi-asserted-by":"publisher","first-page":"1910","DOI":"10.1101\/gad.300822.117.6","volume":"31","author":"Y Hayakawa-Yano","year":"2017","unstructured":"Hayakawa-Yano Y et al (2017) An RNA-binding protein, Qki5, regulates embryonic neural stem cells through pre-mRNA processing in cell adhesion signaling. Genes Dev 31(18):1910\u20131925. https:\/\/doi.org\/10.1101\/gad.300822.117.6","journal-title":"Genes Dev"},{"issue":"10","key":"669_CR69","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1101\/cshperspect.a011544","volume":"4","author":"AG Hinnebusch","year":"2012","unstructured":"Hinnebusch AG, Lorsch JR (2012) The mechanism of eukaryotic translation initiation: New insights and challenges. Cold Spring Harb Perspect Biol 4(10):1\u201325. https:\/\/doi.org\/10.1101\/cshperspect.a011544","journal-title":"Cold Spring Harb Perspect Biol"},{"issue":"1","key":"669_CR70","doi-asserted-by":"publisher","first-page":"118876","DOI":"10.1016\/j.bbamcr.2020.118876","volume":"1868","author":"S Hofmann","year":"2020","unstructured":"Hofmann S, Kedersha N, Anderson P, Ivanov P (2020) Molecular mechanisms of stress granule assembly and disassembly. Biochimica Et Biophysica Acta - Molecular Cell Research 1868(1):118876\u2013118876. https:\/\/doi.org\/10.1016\/j.bbamcr.2020.118876","journal-title":"Biochimica Et Biophysica Acta - Molecular Cell Research"},{"issue":"1","key":"669_CR71","doi-asserted-by":"publisher","first-page":"144","DOI":"10.1016\/j.molcel.2017.09.003","volume":"68","author":"A Hubstenberger","year":"2017","unstructured":"Hubstenberger A et al (2017) P-body purification reveals the condensation of repressed mRNA regulons. Mol Cell 68(1):144-157.e5. https:\/\/doi.org\/10.1016\/j.molcel.2017.09.003","journal-title":"Mol Cell"},{"issue":"12","key":"669_CR72","doi-asserted-by":"publisher","first-page":"6889","DOI":"10.1093\/nar\/gkaa410","volume":"48","author":"J Humphrey","year":"2020","unstructured":"Humphrey J et al (2020) FUS ALS-causative mutations impair FUS autoregulation and splicing factor networks through intron retention. Nucleic Acids Res 48(12):6889\u20136905. https:\/\/doi.org\/10.1093\/nar\/gkaa410","journal-title":"Nucleic Acids Res"},{"issue":"7067","key":"669_CR73","doi-asserted-by":"publisher","first-page":"512","DOI":"10.1038\/nature04115","volume":"438","author":"S Huttelmaier","year":"2005","unstructured":"Huttelmaier S et al (2005) Spatial regulation of beta-actin translation by Src-dependent phosphorylation of ZBP1. Nature 438(7067):512\u2013515. https:\/\/doi.org\/10.1038\/nature04115","journal-title":"Nature"},{"issue":"6","key":"669_CR74","doi-asserted-by":"publisher","first-page":"1067","DOI":"10.1016\/j.neuron.2012.07.009","volume":"75","author":"G Ince-dunn","year":"2012","unstructured":"Ince-dunn G et al (2012) Article neuronal Elav-like ( Hu ) proteins regulate RNA splicing and abundance to control glutamate levels and neuronal excitability. Neuron 75(6):1067\u20131080. https:\/\/doi.org\/10.1016\/j.neuron.2012.07.009","journal-title":"Neuron"},{"key":"669_CR75","doi-asserted-by":"publisher","first-page":"393","DOI":"10.1146\/annurev-cellbio-101011-155831","volume":"30","author":"M Ivshina","year":"2014","unstructured":"Ivshina M, Lasko P, Richter JD (2014) Cytoplasmic polyadenylation element binding proteins in development, health, and disease. Annu Rev Cell Dev Biol 30:393\u2013415. https:\/\/doi.org\/10.1146\/annurev-cellbio-101011-155831","journal-title":"Annu Rev Cell Dev Biol"},{"issue":"3","key":"669_CR76","doi-asserted-by":"publisher","first-page":"487","DOI":"10.1016\/j.cell.2015.12.038","volume":"164","author":"S Jain","year":"2016","unstructured":"Jain S, Wheeler JR, Walters RW, Agrawal A, Barsic A, Parker R (2016) ATPase-modulated stress granules contain a diverse proteome and substructure. Cell 164(3):487\u2013498. https:\/\/doi.org\/10.1016\/j.cell.2015.12.038","journal-title":"Cell"},{"issue":"9","key":"669_CR77","doi-asserted-by":"publisher","first-page":"533","DOI":"10.1038\/nrm4032","volume":"16","author":"E Jankowsky","year":"2015","unstructured":"Jankowsky E, Harris ME (2015) Specificity and nonspecificity in RNA-protein interactions. Nat Rev Mol Cell Biol 16(9):533\u2013544. https:\/\/doi.org\/10.1038\/nrm4032","journal-title":"Nat Rev Mol Cell Biol"},{"issue":"1","key":"669_CR78","doi-asserted-by":"publisher","first-page":"22","DOI":"10.1007\/s12035-013-8427-5","volume":"48","author":"J Janssens","year":"2013","unstructured":"Janssens J et al (2013) Overexpression of ALS-associated p. M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice. Mol Neurobiol 48(1):22\u201335. https:\/\/doi.org\/10.1007\/s12035-013-8427-5","journal-title":"Mol Neurobiol"},{"issue":"4","key":"669_CR79","doi-asserted-by":"publisher","first-page":"905","DOI":"10.1093\/emboj\/cdg089","volume":"22","author":"Y Jin","year":"2003","unstructured":"Jin Y et al (2003) A vertebrate RNA-binding protein Fox-1 regulates tissue-specific splicing via the pentanucleotide GCAUG. EMBO J 22(4):905\u2013912. https:\/\/doi.org\/10.1093\/emboj\/cdg089","journal-title":"EMBO J"},{"issue":"10","key":"669_CR80","doi-asserted-by":"publisher","first-page":"1652","DOI":"10.1038\/s12276-020-00513-7","volume":"52","author":"M Jo","year":"2020","unstructured":"Jo M, Lee S, Jeon YM, Kim S, Kwon Y, Kim HJ (2020) The role of TDP-43 propagation in neurodegenerative diseases: integrating insights from clinical and experimental studies. Exp Mol Med 52(10):1652\u20131662. https:\/\/doi.org\/10.1038\/s12276-020-00513-7","journal-title":"Exp Mol Med"},{"issue":"1","key":"669_CR81","doi-asserted-by":"publisher","first-page":"26","DOI":"10.1016\/j.cell.2014.03.005","volume":"157","author":"H Jung","year":"2014","unstructured":"Jung H, Gkogkas CG, Sonenberg N, Holt CE (2014) Remote control of gene function by local translation. Cell 157(1):26\u201340. https:\/\/doi.org\/10.1016\/j.cell.2014.03.005","journal-title":"Cell"},{"key":"669_CR82","doi-asserted-by":"crossref","unstructured":"Kanopka A, Muhlemann O, Akusjarvi\u00a0G (1996) Inhibition by SR proteins splicing of a regulated adenovirus pre-mRNA. Nature\u00a0381:535\u2013538","DOI":"10.1038\/381535a0"},{"issue":"9","key":"669_CR83","doi-asserted-by":"publisher","first-page":"1193","DOI":"10.1007\/s00439-017-1830-7","volume":"136","author":"K Kapeli","year":"2017","unstructured":"Kapeli K, Martinez FJ, Yeo GW (2017) Genetic mutations in RNA-binding proteins and their roles in ALS. Hum Genet 136(9):1193\u20131214. https:\/\/doi.org\/10.1007\/s00439-017-1830-7","journal-title":"Hum Genet"},{"issue":"6","key":"669_CR84","doi-asserted-by":"publisher","first-page":"1257","DOI":"10.1083\/jcb.151.6.1257","volume":"151","author":"N Kedersha","year":"2000","unstructured":"Kedersha N et al (2000) Dynamic shuttling of TIA-1 accompanies the recruitment of mRNA to mammalian stress granules. J Cell Biol 151(6):1257\u20131268. https:\/\/doi.org\/10.1083\/jcb.151.6.1257","journal-title":"J Cell Biol"},{"issue":"13","key":"669_CR85","doi-asserted-by":"publisher","first-page":"7018","DOI":"10.1073\/pnas.111145598","volume":"98","author":"JD Keene","year":"2001","unstructured":"Keene JD (2001) Ribonucleoprotein infrastructure regulating the flow of genetic information between the genome and the proteome. Proc Natl Acad Sci USA 98(13):7018\u20137024. https:\/\/doi.org\/10.1073\/pnas.111145598","journal-title":"Proc Natl Acad Sci USA"},{"issue":"22","key":"669_CR86","doi-asserted-by":"publisher","first-page":"4845","DOI":"10.1093\/hmg\/dds321","volume":"21","author":"SH Kim","year":"2012","unstructured":"Kim SH, Zhan L, Hanson KA, Tibbetts RS (2012) High-content RNAi screening identifies the type 1 inositol triphosphate receptor as a modifier of TDP-43 localization and neurotoxicity. Hum Mol Genet 21(22):4845\u20134856. https:\/\/doi.org\/10.1093\/hmg\/dds321","journal-title":"Hum Mol Genet"},{"issue":"9","key":"669_CR87","doi-asserted-by":"publisher","first-page":"2945","DOI":"10.1091\/mbc.10.9.2945","volume":"10","author":"M Kohrmann","year":"1999","unstructured":"Kohrmann M, Luo M, Kaether C, DesGroseillers L, Dotti CG, Kiebler MA (1999) Microtubule-dependent recruitment of Staufen-green fluorescent protein into large RNA-containing granules and subsequent dendritic transport in living hippocampal neurons. Mol Biol Cell 10(9):2945\u20132953. https:\/\/doi.org\/10.1091\/mbc.10.9.2945","journal-title":"Mol Biol Cell"},{"issue":"1","key":"669_CR88","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1186\/s13578-021-00577-6","volume":"11","author":"E Kozlov","year":"2021","unstructured":"Kozlov E, Shidlovskii YV, Gilmutdinov R, Schedl P, Zhukova M (2021) The role of CPEB family proteins in the nervous system function in the norm and pathology. Cell Biosci 11(1):1\u201314. https:\/\/doi.org\/10.1186\/s13578-021-00577-6","journal-title":"Cell Biosci"},{"issue":"4","key":"669_CR89","doi-asserted-by":"publisher","first-page":"683","DOI":"10.1016\/s0896-6273(01)00508-6","volume":"32","author":"AM Krichevsky","year":"2001","unstructured":"Krichevsky AM, Kosik KS (2001) Neuronal RNA granules: a link between RNA localization and stimulation-dependent translation. Neuron 32(4):683\u2013696. https:\/\/doi.org\/10.1016\/s0896-6273(01)00508-6","journal-title":"Neuron"},{"issue":"7","key":"669_CR90","doi-asserted-by":"publisher","first-page":"4712","DOI":"10.1093\/nar\/gkt1407","volume":"42","author":"PH Kuo","year":"2014","unstructured":"Kuo PH, Chiang CH, Wang YT, Doudeva LG, Yuan HS (2014) The crystal structure of TDP-43 RRM1-DNA complex reveals the specific recognition for UG- and TG-rich nucleic acids. Nucleic Acids Res 42(7):4712\u20134722. https:\/\/doi.org\/10.1093\/nar\/gkt1407","journal-title":"Nucleic Acids Res"},{"issue":"4","key":"669_CR91","doi-asserted-by":"publisher","first-page":"785","DOI":"10.1002\/jnr.21534","volume":"86","author":"TL Lauriat","year":"2008","unstructured":"Lauriat TL et al (2008) Developmental expression profile of Quaking, a candidate gene for schizophrenia, and its target genes in human prefrontal cortex and hippocampus shows regional specificity. J Neurosci Res 86(4):785\u2013796. https:\/\/doi.org\/10.1002\/jnr.21534","journal-title":"J Neurosci Res"},{"issue":"1","key":"669_CR92","doi-asserted-by":"publisher","first-page":"113","DOI":"10.1016\/j.neuron.2015.11.025.Cytoplasmic","volume":"89","author":"J-A Lee","year":"2016","unstructured":"Lee J-A et al (2016) Cytoplasmic Rbfox1 regulates the expression of synaptic and autism-related genes. Neuron 89(1):113\u2013128. https:\/\/doi.org\/10.1016\/j.neuron.2015.11.025.Cytoplasmic","journal-title":"Neuron"},{"key":"669_CR93","doi-asserted-by":"publisher","unstructured":"Lee Y, Rio DC, Biology S, Biology\u00a0C (2015) Mechanisms and Regulation of Alternative Pre-mRNA Splicing Yeon. Annu Rev Biochem\u00a0(258):291\u2013323. https:\/\/doi.org\/10.1146\/annurev-biochem-060614-034316.Mechanisms","DOI":"10.1146\/annurev-biochem-060614-034316.Mechanisms"},{"issue":"3","key":"669_CR94","doi-asserted-by":"publisher","first-page":"323","DOI":"10.1016\/s0092-8674(00)80668-6","volume":"100","author":"HA Lewis","year":"2000","unstructured":"Lewis HA et al (2000) Sequence-specific RNA binding by a Nova KH domain: implications for paraneoplastic disease and the fragile X syndrome. Cell 100(3):323\u2013332. https:\/\/doi.org\/10.1016\/s0092-8674(00)80668-6","journal-title":"Cell"},{"issue":"7221","key":"669_CR95","doi-asserted-by":"publisher","first-page":"464","DOI":"10.1038\/nature07488","volume":"456","author":"DD Licatalosi","year":"2008","unstructured":"Licatalosi DD et al (2008) HITS-CLIP yields genome-wide insights into brain alternative RNA processing. Nature 456(7221):464\u2013469. https:\/\/doi.org\/10.1038\/nature07488","journal-title":"Nature"},{"key":"669_CR96","doi-asserted-by":"publisher","unstructured":"Lukong KE, Chang KW, Khandjian EW, Richard\u00a0S 2008) RNA-binding proteins in human genetic disease. Trends Gen 24(8):416\u2013425. https:\/\/doi.org\/10.1016\/j.tig.2008.05.004","DOI":"10.1016\/j.tig.2008.05.004"},{"issue":"6","key":"669_CR97","doi-asserted-by":"publisher","first-page":"479","DOI":"10.1038\/nrm2178","volume":"8","author":"BM Lunde","year":"2007","unstructured":"Lunde BM, Moore C, Varani G (2007) RNA-binding proteins: Modular design for efficient function. Nat Rev Mol Cell Biol 8(6):479\u2013490. https:\/\/doi.org\/10.1038\/nrm2178","journal-title":"Nat Rev Mol Cell Biol"},{"key":"669_CR98","doi-asserted-by":"crossref","unstructured":"Malik AM, Miguez RA, Li X, Ho Y-S, L EF, Barmada\u00a0SJ (2018) Matrin 3-dependent neurotoxicity is modified by nucleic acid binding and nucleocytoplasmic localization. BioRxiv 1\u201352","DOI":"10.7554\/eLife.35977.024"},{"key":"669_CR99","doi-asserted-by":"publisher","unstructured":"Mann\u00a0JR et al\u00a0(2019) RNA binding antagonizes neurotoxic phase transitions of TDP-43. Neuron 102(2):321\u2013338 e8. https:\/\/doi.org\/10.1016\/j.neuron.2019.01.048","DOI":"10.1016\/j.neuron.2019.01.048"},{"issue":"9","key":"669_CR100","doi-asserted-by":"publisher","first-page":"2118","DOI":"10.1111\/j.1742-4658.2005.04653.x","volume":"272","author":"C Maris","year":"2005","unstructured":"Maris C, Dominguez C, Allain FHT (2005) The RNA recognition motif, a plastic RNA-binding platform to regulate post-transcriptional gene expression. FEBS J 272(9):2118\u20132131. https:\/\/doi.org\/10.1111\/j.1742-4658.2005.04653.x","journal-title":"FEBS J"},{"issue":"3","key":"669_CR101","doi-asserted-by":"publisher","first-page":"590","DOI":"10.1016\/j.cell.2017.12.032","volume":"172","author":"S Markmiller","year":"2018","unstructured":"Markmiller S et al (2018) Context-dependent and disease-specific diversity in protein interactions within stress granules. Cell 172(3):590-604.e13. https:\/\/doi.org\/10.1016\/j.cell.2017.12.032","journal-title":"Cell"},{"issue":"4","key":"669_CR102","doi-asserted-by":"publisher","first-page":"719","DOI":"10.1016\/j.cell.2009.01.044","volume":"136","author":"KC Martin","year":"2009","unstructured":"Martin KC, Ephrussi A (2009) mRNA localization: gene expression in the spatial dimension. Cell 136(4):719\u2013730. https:\/\/doi.org\/10.1016\/j.cell.2009.01.044","journal-title":"Cell"},{"issue":"87","key":"669_CR103","doi-asserted-by":"publisher","first-page":"1","DOI":"10.3791\/51197","volume":"43","author":"S Martin","year":"2014","unstructured":"Martin S, Tazi J (2014) Visualization of G3BP stress granules dynamics in live primary cells. J vis Exp 43(87):1\u20138. https:\/\/doi.org\/10.3791\/51197","journal-title":"J vis Exp"},{"issue":"2","key":"669_CR104","doi-asserted-by":"publisher","first-page":"172","DOI":"10.1371\/journal.pbio.0040021","volume":"4","author":"R Martinez-Contreras","year":"2006","unstructured":"Martinez-Contreras R, Fisette JF, Nasim FUH, Madden R, Cordeau M, Chabot B (2006) Intronic binding sites for hnRNP A\/B and hnRNP F\/H proteins stimulate pre-mRNA splicing. PLoS Biol 4(2):172\u2013185. https:\/\/doi.org\/10.1371\/journal.pbio.0040021","journal-title":"PLoS Biol"},{"issue":"10","key":"669_CR105","doi-asserted-by":"publisher","first-page":"1045","DOI":"10.1101\/gad.255737.114","volume":"29","author":"A Masuda","year":"2015","unstructured":"Masuda A et al (2015) Position-specific binding of FUS to nascent RNA regulates mRNA length. Genes Dev 29(10):1045\u20131057. https:\/\/doi.org\/10.1101\/gad.255737.114","journal-title":"Genes Dev"},{"key":"669_CR106","doi-asserted-by":"crossref","unstructured":"Matoulkova E, Michalova E, Vojtesek B, Hrstka\u00a0AR (2012) The role of the 3' untranslated region in post-transcriptional regulation of protein expression in mammalian cells. RNA Biol 9(5):563\u2013576","DOI":"10.4161\/rna.20231"},{"key":"669_CR107","doi-asserted-by":"publisher","unstructured":"Moore\u00a0KS, von Lindern\u00a0M (2018) RNA binding proteins and regulation of mRNA translation in erythropoiesis. Front Physiol\u00a09:1\u201317. https:\/\/doi.org\/10.3389\/fphys.2018.00910","DOI":"10.3389\/fphys.2018.00910"},{"key":"669_CR108","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1007\/s00401-019-01999-w","volume":"138","author":"S Moore","year":"2019","unstructured":"Moore S et al (2019) ADAR2 mislocalization and widespread RNA editing aberrations in C9orf72-mediated ALS\/FTD. Acta Neuropathol 138:49\u201365. https:\/\/doi.org\/10.1007\/s00401-019-01999-w","journal-title":"Acta Neuropathol"},{"key":"669_CR109","doi-asserted-by":"publisher","unstructured":"Neelagandan N, Lamberti I, Carvalho HJF, Gobet C, Naef\u00a0F (2020) What determines eukaryotic translation elongation: Recent molecular and quantitative analyses of protein synthesis: Determinants of eukaryotic translation.\u00a0Open Biol\u00a010(12). https:\/\/doi.org\/10.1098\/rsob.200292rsob200292","DOI":"10.1098\/rsob.200292rsob200292"},{"issue":"5796","key":"669_CR110","doi-asserted-by":"publisher","first-page":"130","DOI":"10.1126\/science.1134108","volume":"314","author":"M Neumann","year":"2006","unstructured":"Neumann M et al (2006) Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314(5796):130\u2013133. https:\/\/doi.org\/10.1126\/science.1134108","journal-title":"Science"},{"key":"669_CR111","doi-asserted-by":"publisher","first-page":"1385","DOI":"10.1091\/mbc.E06","volume":"18","author":"Nonhoff","year":"2007","unstructured":"Nonhoff et al (2007) Ataxin-2 interacts with the DEAD\/H-Box RNA Helicase DDX6 and interferes with P-bodies and stress granules. Mol Biol Cell 18:1385\u20131396. https:\/\/doi.org\/10.1091\/mbc.E06","journal-title":"Mol Biol Cell"},{"issue":"10","key":"669_CR112","doi-asserted-by":"publisher","first-page":"643","DOI":"10.1038\/nrm3645","volume":"14","author":"CJ Norbury","year":"2013","unstructured":"Norbury CJ (2013) Cytoplasmic RNA: A case of the tail wagging the dog. Nat Rev Mol Cell Biol 14(10):643\u2013653. https:\/\/doi.org\/10.1038\/nrm3645","journal-title":"Nat Rev Mol Cell Biol"},{"issue":"1","key":"669_CR113","doi-asserted-by":"publisher","first-page":"22","DOI":"10.1590\/1678-4685-gmb-2016-0258","volume":"40","author":"C Oliveira","year":"2017","unstructured":"Oliveira C, Faoro H, Alves LR, Goldenberg S (2017) RNA-binding proteins and their role in the regulation of gene expression in trypanosoma cruzi and saccharomyces cerevisiae. Genet Mol Biol 40(1):22\u201330. https:\/\/doi.org\/10.1590\/1678-4685-gmb-2016-0258","journal-title":"Genet Mol Biol"},{"issue":"6","key":"669_CR114","doi-asserted-by":"publisher","first-page":"2","DOI":"10.3390\/genes8060157","volume":"8","author":"LA Ostrowski","year":"2017","unstructured":"Ostrowski LA, Hall AC, Mekhail K (2017) Ataxin-2: from RNA control to human health and disease. Genes 8(6):2\u201321. https:\/\/doi.org\/10.3390\/genes8060157","journal-title":"Genes"},{"key":"669_CR115","doi-asserted-by":"crossref","unstructured":"Oubridge C, Ito N, Evans PR, Teo CH, Nagai\u00a0K (1994) Crystal structure at 1.92 \u00c5 resolution of the RNA-binding domain of the U1A spliceosomal protein complexed with an RNA hairpin. Nature\u00a0372:432\u2013438","DOI":"10.1038\/372432a0"},{"issue":"1","key":"669_CR116","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1186\/s12864-020-07291-6","volume":"21","author":"X Pan","year":"2020","unstructured":"Pan X, Fang Y, Li X, Yang Y, Shen HB (2020) RBPsuite: RNA-protein binding sites prediction suite based on deep learning. BMC Genomics 21(1):1\u20138. https:\/\/doi.org\/10.1186\/s12864-020-07291-6","journal-title":"BMC Genomics"},{"issue":"3","key":"669_CR117","doi-asserted-by":"publisher","first-page":"313","DOI":"10.1083\/jcb.201609081","volume":"215","author":"MD Panas","year":"2016","unstructured":"Panas MD, Ivanov P, Anderson P (2016) Mechanistic insights into mammalian stress granule dynamics. J Cell Biol 215(3):313\u2013323. https:\/\/doi.org\/10.1083\/jcb.201609081","journal-title":"J Cell Biol"},{"issue":"28","key":"669_CR118","doi-asserted-by":"publisher","first-page":"25301","DOI":"10.1074\/jbc.M110.206540","volume":"286","author":"E Park","year":"2011","unstructured":"Park E et al (2011) Regulatory roles of heterogeneous nuclear ribonucleoprotein M and Nova-1 protein in alternative splicing of dopamine D2 receptor pre-mRNA. J Biol Chem 286(28):25301\u201325308. https:\/\/doi.org\/10.1074\/jbc.M110.206540","journal-title":"J Biol Chem"},{"issue":"5","key":"669_CR119","doi-asserted-by":"publisher","first-page":"635","DOI":"10.1016\/j.molcel.2007.02.011","volume":"25","author":"R Parker","year":"2007","unstructured":"Parker R, Sheth U (2007) P bodies and the control of mRNA translation and degradation. Mol Cell 25(5):635\u2013646. https:\/\/doi.org\/10.1016\/j.molcel.2007.02.011","journal-title":"Mol Cell"},{"issue":"7719","key":"669_CR120","doi-asserted-by":"publisher","first-page":"441","DOI":"10.1038\/s41586-018-0423-5","volume":"560","author":"A Parras","year":"2018","unstructured":"Parras A et al (2018) Autism-like phenotype and risk gene mRNA deadenylation by CPEB4 mis-splicing. Genes Dev 560(7719):441\u2013446. https:\/\/doi.org\/10.1038\/s41586-018-0423-5","journal-title":"Genes Dev"},{"issue":"R2","key":"669_CR121","doi-asserted-by":"publisher","first-page":"156","DOI":"10.1093\/hmg\/ddp303","volume":"18","author":"GS Pesiridis","year":"2009","unstructured":"Pesiridis GS, Lee VMY, Trojanowski JQ (2009) Mutations in TDP-43 link glycine-rich domain functions to amyotrophic lateral sclerosis. Hum Mol Genet 18(R2):156\u2013162. https:\/\/doi.org\/10.1093\/hmg\/ddp303","journal-title":"Hum Mol Genet"},{"issue":"5","key":"669_CR122","doi-asserted-by":"publisher","first-page":"501","DOI":"10.1002\/wrna.1289","volume":"6","author":"LJ Pilaz","year":"2015","unstructured":"Pilaz LJ, Silver DL (2015) Post-transcriptional regulation in corticogenesis: How RNA-binding proteins help build the brain. Wiley Interdisciplinary Reviews: RNA 6(5):501\u2013515. https:\/\/doi.org\/10.1002\/wrna.1289","journal-title":"Wiley Interdisciplinary Reviews: RNA"},{"issue":"7739","key":"669_CR123","doi-asserted-by":"publisher","first-page":"356","DOI":"10.1038\/s41586-018-0847-y","volume":"565","author":"A Poulopoulos","year":"2019","unstructured":"Poulopoulos A et al (2019) Subcellular transcriptomes and proteomes of developing axon projections in the cerebral cortex. Nature 565(7739):356\u2013360. https:\/\/doi.org\/10.1038\/s41586-018-0847-y","journal-title":"Nature"},{"issue":"1","key":"669_CR124","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1016\/j.neuron.2015.05.004","volume":"87","author":"B Raj","year":"2015","unstructured":"Raj B, Blencowe BJ (2015) Alternative Splicing in the Mammalian Nervous System: Recent Insights into Mechanisms and Functional Roles. Neuron 87(1):14\u201327. https:\/\/doi.org\/10.1016\/j.neuron.2015.05.004","journal-title":"Neuron"},{"issue":"12","key":"669_CR125","doi-asserted-by":"publisher","first-page":"1770","DOI":"10.15252\/embj.201695748","volume":"36","author":"M Rajman","year":"2017","unstructured":"Rajman M et al (2017) A microRNA-129-5p\/Rbfox crosstalk coordinates homeostatic downscaling of excitatory synapses. EMBO J 36(12):1770\u20131787. https:\/\/doi.org\/10.15252\/embj.201695748","journal-title":"EMBO J"},{"key":"669_CR126","doi-asserted-by":"crossref","unstructured":"Rayman\u00a0JB, Kandel\u00a0ER (2017) TIA-1 is a functional prion-like protein. 1\u201313","DOI":"10.1101\/cshperspect.a030718"},{"issue":"6","key":"669_CR127","doi-asserted-by":"publisher","first-page":"279","DOI":"10.1016\/j.tibs.2007.04.004","volume":"32","author":"JD Richter","year":"2007","unstructured":"Richter JD (2007) CPEB: a life in translation. Trends Biochem Sci 32(6):279\u2013285. https:\/\/doi.org\/10.1016\/j.tibs.2007.04.004","journal-title":"Trends Biochem Sci"},{"issue":"1","key":"669_CR128","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1186\/s13059-017-1330-z","volume":"18","author":"OS Rissland","year":"2017","unstructured":"Rissland OS et al (2017) The influence of microRNAs and poly(A) tail length on endogenous mRNA-protein complexes. Genome Biol 18(1):1\u201318. https:\/\/doi.org\/10.1186\/s13059-017-1330-z","journal-title":"Genome Biol"},{"key":"669_CR129","doi-asserted-by":"publisher","unstructured":"Robberecht W, Eykens\u00a0C (2015) The genetic basis of amyotrophic lateral sclerosis: recent breakthroughs. Adv Genom Gen\u00a0327\u2013327. https:\/\/doi.org\/10.2147\/agg.s57397","DOI":"10.2147\/agg.s57397"},{"issue":"3","key":"669_CR130","doi-asserted-by":"publisher","first-page":"1309","DOI":"10.1152\/physrev.00047.2019","volume":"101","author":"R Schieweck","year":"2021","unstructured":"Schieweck R, Ninkovic J, Kiebler MA (2021) RNA-binding proteins balance brain function in health and disease. Physiol Rev 101(3):1309\u20131370. https:\/\/doi.org\/10.1152\/physrev.00047.2019","journal-title":"Physiol Rev"},{"issue":"2","key":"669_CR131","doi-asserted-by":"publisher","first-page":"77","DOI":"10.2174\/138920210790886844","volume":"11","author":"B Schoser","year":"2010","unstructured":"Schoser B, Timchenko L (2010) Myotonic dystrophies 1 and 2: complex diseases with complex mechanisms. Curr Genomics 11(2):77\u201390. https:\/\/doi.org\/10.2174\/138920210790886844","journal-title":"Curr Genomics"},{"issue":"1","key":"669_CR132","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1080\/10409238.2020.1841726","volume":"56","author":"JL Schwartz","year":"2021","unstructured":"Schwartz JL, Jones KL, Yeo GW (2021) Repeat RNA expansion disorders of the nervous system: post-transcriptional mechanisms and therapeutic strategies. Crit Rev Biochem Mol Biol 56(1):31\u201353. https:\/\/doi.org\/10.1080\/10409238.2020.1841726","journal-title":"Crit Rev Biochem Mol Biol"},{"key":"669_CR133","doi-asserted-by":"crossref","unstructured":"Selvanathan SP, Graham GT, Erkizan HV, Dirsken U, Natarajan T, and Dakic A (2015) Oncogenic fusion protein EWS-FLI1 is a network hub that regulates alternative splicing. PNAS 12(11)","DOI":"10.1073\/pnas.1500536112"},{"key":"669_CR134","doi-asserted-by":"publisher","unstructured":"Sena RM, Twiss JL, Gardiner AS, Dell\u2019orco M, Linsenbardt DN, Perrone-Bizzozero\u00a0NI (2021) The RNA-binding protein HuD regulates alternative splicing and alternative polyadenylation in the mouse neocortex. Molecules\u00a026(10). https:\/\/doi.org\/10.3390\/molecules26102836","DOI":"10.3390\/molecules26102836"},{"issue":"1\u20132","key":"669_CR135","doi-asserted-by":"publisher","first-page":"139","DOI":"10.1016\/s0006-8993(97)00921-9","volume":"773","author":"P Shashidharan","year":"1997","unstructured":"Shashidharan P et al (1997) Immunohistochemical localization of the neuron-specific glutamate transporter EAAC1 (EAAT3) in rat brain and spinal cord revealed by a novel monoclonal antibody. Brain Res 773(1\u20132):139\u2013148. https:\/\/doi.org\/10.1016\/s0006-8993(97)00921-9","journal-title":"Brain Res"},{"issue":"6","key":"669_CR136","doi-asserted-by":"publisher","first-page":"885","DOI":"10.1093\/jb\/mvq025","volume":"147","author":"DJ Shaw","year":"2010","unstructured":"Shaw DJ, Morse R, Todd AG, Eggleton P, Lorson CL, Young PJ (2010) Identification of a self-association domain in the Ewing\u2019s sarcoma protein: a novel function for arginine-glycine-glycine rich motifs? J Biochem 147(6):885\u2013893. https:\/\/doi.org\/10.1093\/jb\/mvq025","journal-title":"J Biochem"},{"issue":"1","key":"669_CR137","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1002\/wrna.1573","volume":"11","author":"CR Shotwell","year":"2020","unstructured":"Shotwell CR, Cleary JD, Berglund JA (2020) The potential of engineered eukaryotic RNA-binding proteins as molecular tools and therapeutics. Wiley Interdisciplinary Reviews: RNA 11(1):1\u201321. https:\/\/doi.org\/10.1002\/wrna.1573","journal-title":"Wiley Interdisciplinary Reviews: RNA"},{"issue":"7","key":"669_CR138","doi-asserted-by":"publisher","first-page":"615","DOI":"10.1016\/j.molmed.2016.05.005","volume":"22","author":"S Shukla","year":"2016","unstructured":"Shukla S, Parker R (2016) Hypo- and hyper-assembly diseases of RNA\u2013protein complexes. Trends Mol Med 22(7):615\u2013628. https:\/\/doi.org\/10.1016\/j.molmed.2016.05.005","journal-title":"Trends Mol Med"},{"key":"669_CR139","doi-asserted-by":"publisher","unstructured":"Sibley CR, Blazquez L, Ule\u00a0J (2016) Lessons from non-canonical splicing Christopher. 17(7)407\u2013421. https:\/\/doi.org\/10.1038\/nrg.2016.46.Lessons","DOI":"10.1038\/nrg.2016.46.Lessons"},{"issue":"February","key":"669_CR140","doi-asserted-by":"publisher","first-page":"325","DOI":"10.1146\/annurev-biochem-080111-092106","volume":"84","author":"G Singh","year":"2015","unstructured":"Singh G, Pratt G, Yeo GW, Moore MJ (2015) The clothes make the mRNA: past and present trends in mRNP fashion. Annu Rev Biochem 84(February):325\u2013354. https:\/\/doi.org\/10.1146\/annurev-biochem-080111-092106","journal-title":"Annu Rev Biochem"},{"issue":"8","key":"669_CR141","doi-asserted-by":"publisher","first-page":"472","DOI":"10.1016\/j.molmed.2012.06.006","volume":"18","author":"RK Singh","year":"2012","unstructured":"Singh RK, Cooper TA (2012) Pre-mRNA splicing in disease and therapeutics. Trends Mol Med 18(8):472\u2013482. https:\/\/doi.org\/10.1016\/j.molmed.2012.06.006","journal-title":"Trends Mol Med"},{"issue":"1","key":"669_CR142","doi-asserted-by":"publisher","first-page":"202","DOI":"10.1080\/19491034.2018.1439304","volume":"9","author":"M Soheilypour","year":"2018","unstructured":"Soheilypour M, Mofrad MRK (2018) Quality control of mRNAs at the entry of the nuclear pore: cooperation in a complex molecular system. Nucleus 9(1):202\u2013211. https:\/\/doi.org\/10.1080\/19491034.2018.1439304","journal-title":"Nucleus"},{"issue":"4","key":"669_CR143","doi-asserted-by":"publisher","first-page":"731","DOI":"10.1016\/j.cell.2009.01.042.Regulation","volume":"136","author":"N Sonenberg","year":"2009","unstructured":"Sonenberg N, Hinnebusch AG (2009) Regulation of translation initiation in eukaryotes. Cell 136(4):731\u2013745. https:\/\/doi.org\/10.1016\/j.cell.2009.01.042.Regulation","journal-title":"Cell"},{"issue":"5","key":"669_CR144","doi-asserted-by":"publisher","first-page":"1001","DOI":"10.1242\/jcs.161679","volume":"128","author":"T Song","year":"2015","unstructured":"Song T et al (2015) Specific interaction of KIF11 with ZBP1 regulates the transport of beta-actin mRNA and cell motility. J Cell Sci 128(5):1001\u20131010. https:\/\/doi.org\/10.1242\/jcs.161679","journal-title":"J Cell Sci"},{"issue":"5870","key":"669_CR145","doi-asserted-by":"publisher","first-page":"1668","DOI":"10.1126\/science.1154584","volume":"319","author":"J Sreedharan","year":"2008","unstructured":"Sreedharan J et al (2008) TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis. Science 319(5870):1668\u20131672. https:\/\/doi.org\/10.1126\/science.1154584","journal-title":"Science"},{"issue":"1","key":"669_CR146","doi-asserted-by":"publisher","first-page":"33","DOI":"10.1038\/sj.embor.7400325","volume":"6","author":"R Stefl","year":"2005","unstructured":"Stefl R, Skrisovska L, Allain FHT (2005) RNA sequence- and shape-dependent recognition by proteins in the ribonucleoprotein particle. EMBO Rep 6(1):33\u201338. https:\/\/doi.org\/10.1038\/sj.embor.7400325","journal-title":"EMBO Rep"},{"key":"669_CR147","unstructured":"Suresh S, Joladarashi D, Jeyabal P, Amirthalingam R, Krishnamurthy\u00a0TA (2016) RNA stabilizing proteins as molecular targets in cardiovascular pathologies. Trends Cardiovasc Med 176(1):139\u2013148"},{"issue":"1","key":"669_CR148","doi-asserted-by":"publisher","first-page":"58","DOI":"10.1093\/bfgp\/els056","volume":"12","author":"E Szostak","year":"2013","unstructured":"Szostak E, Gebauer F (2013) Translational control by 3\u2019-UTR-binding proteins. Brief Funct Genomics 12(1):58\u201365. https:\/\/doi.org\/10.1093\/bfgp\/els056","journal-title":"Brief Funct Genomics"},{"issue":"March","key":"669_CR149","doi-asserted-by":"publisher","first-page":"1","DOI":"10.3389\/fnmol.2019.00051","volume":"12","author":"M Telias","year":"2019","unstructured":"Telias M (2019) Molecular mechanisms of synaptic dysregulation in fragile X syndrome and autism spectrum disorders. Front Mol Neurosci 12(March):1\u201312. https:\/\/doi.org\/10.3389\/fnmol.2019.00051","journal-title":"Front Mol Neurosci"},{"key":"669_CR150","doi-asserted-by":"crossref","unstructured":"Terenzio\u00a0M et al (2018) Locally translated mTOR controls axonal local translation in nerve injury. 1421:1416\u20131421","DOI":"10.1126\/science.aan1053"},{"issue":"Pt 4","key":"669_CR151","doi-asserted-by":"publisher","first-page":"1003","DOI":"10.1242\/jcs.096149","volume":"125","author":"K Thelen","year":"2012","unstructured":"Thelen K, Maier B, Faber M, Albrecht C, Fischer P, Pollerberg GE (2012) Translation of the cell adhesion molecule ALCAM in axonal growth cones - regulation and functional importance. J Cell Sci 125(Pt 4):1003\u20131014. https:\/\/doi.org\/10.1242\/jcs.096149","journal-title":"J Cell Sci"},{"issue":"January","key":"669_CR152","doi-asserted-by":"publisher","first-page":"1","DOI":"10.3389\/fmolb.2019.00161","volume":"6","author":"MP Thelen","year":"2020","unstructured":"Thelen MP, Kye MJ (2020) The role of RNA binding proteins for local mRNA translation: implications in neurological disorders. Front Mol Biosci 6(January):1\u201313. https:\/\/doi.org\/10.3389\/fmolb.2019.00161","journal-title":"Front Mol Biosci"},{"issue":"8","key":"669_CR153","doi-asserted-by":"publisher","first-page":"3251","DOI":"10.1523\/jneurosci.23-08-03251.2003","volume":"23","author":"DM Tiruchinapalli","year":"2003","unstructured":"Tiruchinapalli DM et al (2003) Activity-dependent trafficking and dynamic localization of zipcode binding protein 1 and \u03b2-actin mRNA in dendrites and spines of hippocampal neurons. J Neurosci 23(8):3251\u20133261. https:\/\/doi.org\/10.1523\/jneurosci.23-08-03251.2003","journal-title":"J Neurosci"},{"issue":"3","key":"669_CR154","doi-asserted-by":"publisher","first-page":"883","DOI":"10.1093\/molbev\/msr263","volume":"29","author":"M Toll-Riera","year":"2012","unstructured":"Toll-Riera M, Rad\u00f3-Trilla N, Martys F, Alb\u00e1 MM (2012) Role of low-complexity sequences in the formation of novel protein coding sequences. Mol Biol Evol 29(3):883\u2013886. https:\/\/doi.org\/10.1093\/molbev\/msr263","journal-title":"Mol Biol Evol"},{"key":"669_CR155","doi-asserted-by":"publisher","unstructured":"Turner-Bridger B, Caterino C, Cioni\u00a0JM (2020) Molecular mechanisms behind mRNA localization in axons: axonal mRNA localisation. Open Biol\u00a010(9). https:\/\/doi.org\/10.1098\/rsob.200177","DOI":"10.1098\/rsob.200177"},{"issue":"1","key":"669_CR156","doi-asserted-by":"publisher","first-page":"1","DOI":"10.4161\/pri.5.1.14265","volume":"5","author":"M Udan","year":"2011","unstructured":"Udan M, Baloh RH (2011) Implications of the prion-related Q\/N domains in TDP-43 and FUS. Prion 5(1):1\u20135. https:\/\/doi.org\/10.4161\/pri.5.1.14265","journal-title":"Prion"},{"issue":"7119","key":"669_CR157","doi-asserted-by":"publisher","first-page":"580","DOI":"10.1038\/nature05304","volume":"444","author":"J Ule","year":"2006","unstructured":"Ule J et al (2006) An RNA map predicting Nova-dependent splicing regulation. Nature 444(7119):580\u2013586. https:\/\/doi.org\/10.1038\/nature05304","journal-title":"Nature"},{"key":"669_CR158","doi-asserted-by":"publisher","unstructured":"Uversky\u00a0VN (2019) Intrinsically disordered proteins and their \u201cMysterious\u201d (meta)physics. Front Phys 7:8\u201323. https:\/\/doi.org\/10.3389\/fphy.2019.00010","DOI":"10.3389\/fphy.2019.00010"},{"issue":"11","key":"669_CR159","doi-asserted-by":"publisher","first-page":"2712","DOI":"10.1111\/j.1742-4658.2008.06411.x","volume":"275","author":"R Valverde","year":"2008","unstructured":"Valverde R, Edwards L, Regan L (2008) Structure and function of KH domains. FEBS J 275(11):2712\u20132726. https:\/\/doi.org\/10.1111\/j.1742-4658.2008.06411.x","journal-title":"FEBS J"},{"issue":"7818","key":"669_CR160","doi-asserted-by":"publisher","first-page":"711","DOI":"10.1038\/s41586-020-2077-3","volume":"583","author":"EL Van Nostrand","year":"2020","unstructured":"Van Nostrand EL et al (2020) A large-scale binding and functional map of human RNA-binding proteins. Nature 583(7818):711\u2013719. https:\/\/doi.org\/10.1038\/s41586-020-2077-3","journal-title":"Nature"},{"issue":"5918","key":"669_CR161","doi-asserted-by":"publisher","first-page":"1208","DOI":"10.1126\/science.1165942","volume":"323","author":"C Vance","year":"2009","unstructured":"Vance C et al (2009) Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Science 323(5918):1208\u20131211. https:\/\/doi.org\/10.1126\/science.1165942","journal-title":"Science"},{"issue":"6","key":"669_CR162","doi-asserted-by":"publisher","first-page":"524","DOI":"10.1159\/000354170","volume":"59","author":"T Vanderweyde","year":"2013","unstructured":"Vanderweyde T, Youmans K, Liu-Yesucevitz L, Wolozin B (2013) Role of stress granules and RNA-binding proteins in neurodegeneration: a mini-review. Gerontology 59(6):524\u2013533. https:\/\/doi.org\/10.1159\/000354170","journal-title":"Gerontology"},{"key":"669_CR163","doi-asserted-by":"publisher","first-page":"407","DOI":"10.1146\/annurev.biophys.27.1.407","volume":"27","author":"G Varani","year":"1998","unstructured":"Varani G, Nagai K (1998) RNA recognition by RNP proteins during RNA processing. Annu Rev Biophys Biomol Struct 27:407\u2013445. https:\/\/doi.org\/10.1146\/annurev.biophys.27.1.407","journal-title":"Annu Rev Biophys Biomol Struct"},{"issue":"9","key":"669_CR164","doi-asserted-by":"publisher","first-page":"5949","DOI":"10.1093\/nar\/gku193","volume":"42","author":"I Wang","year":"2014","unstructured":"Wang I, Hennig J, Jagtap PKA, Sonntag M, Valc\u00e1rcel J, Sattler M (2014) Structure, dynamics and RNA binding of the multi-domain splicing factor TIA-1. Nucleic Acids Res 42(9):5949\u20135966. https:\/\/doi.org\/10.1093\/nar\/gku193","journal-title":"Nucleic Acids Res"},{"issue":"2","key":"669_CR165","doi-asserted-by":"publisher","first-page":"152","DOI":"10.3892\/br.2014.407","volume":"3","author":"Wang","year":"2015","unstructured":"Wang et al (2015) Mechanism of alternative splicing and its regulation. Biomedical Reports 3(2):152\u2013158. https:\/\/doi.org\/10.3892\/br.2014.407","journal-title":"Biomedical Reports"},{"issue":"45","key":"669_CR166","doi-asserted-by":"publisher","first-page":"11418","DOI":"10.1523\/JNEUROSCI.2352-16.2016","volume":"36","author":"ET Wang","year":"2016","unstructured":"Wang ET et al (2016) Dysregulation of mRNA localization and translation in genetic disease. J Neurosci 36(45):11418\u201311426. https:\/\/doi.org\/10.1523\/JNEUROSCI.2352-16.2016","journal-title":"J Neurosci"},{"issue":"3","key":"669_CR167","doi-asserted-by":"publisher","first-page":"1995","DOI":"10.1002\/jcp.29186","volume":"235","author":"Z Wang","year":"2019","unstructured":"Wang Z et al (2019) The emerging roles of hnRNPK. J Cell Physiol 235(3):1995\u20132008. https:\/\/doi.org\/10.1002\/jcp.29186","journal-title":"J Cell Physiol"},{"issue":"1","key":"669_CR168","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1186\/s40478-020-01055-9","volume":"8","author":"R Watanabe","year":"2020","unstructured":"Watanabe R et al (2020) Intracellular dynamics of Ataxin-2 in the human brains with normal and frontotemporal lobar degeneration with TDP-43 inclusions. Acta Neuropathol Commun 8(1):1\u201319. https:\/\/doi.org\/10.1186\/s40478-020-01055-9","journal-title":"Acta Neuropathol Commun"},{"issue":"44","key":"669_CR169","doi-asserted-by":"publisher","first-page":"18809","DOI":"10.1073\/pnas.0908767106","volume":"106","author":"I Wegorzewska","year":"2009","unstructured":"Wegorzewska I, Bell S, Cairns NJ, Miller TM, Baloh RH (2009) TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration. Proc Natl Acad Sci USA 106(44):18809\u201318814. https:\/\/doi.org\/10.1073\/pnas.0908767106","journal-title":"Proc Natl Acad Sci USA"},{"key":"669_CR170","doi-asserted-by":"publisher","first-page":"183","DOI":"10.1146\/annurev.biophys.29.1.183","volume":"3","author":"SA Wolfe","year":"2000","unstructured":"Wolfe SA, Nekludova L, Pabo CO (2000) DNA RECOGNITION BY Cys2His2 ZINC FINGER PROTEINS. Annual Review of Biophysics and Biomolecular Structure Rev Biophys Biomol Struct 3:183\u2013212","journal-title":"Annual Review of Biophysics and Biomolecular Structure Rev Biophys Biomol Struct"},{"issue":"11","key":"669_CR171","doi-asserted-by":"publisher","first-page":"649","DOI":"10.1038\/s41583-019-0222-5.Stress","volume":"20","author":"Wolozin and Ivanov","year":"2019","unstructured":"Wolozin and Ivanov (2019) Stress granules and neurodegeneration. Nat Rev Neurosci 20(11):649\u2013666. https:\/\/doi.org\/10.1038\/s41583-019-0222-5.Stress","journal-title":"Nat Rev Neurosci"},{"issue":"6","key":"669_CR172","doi-asserted-by":"publisher","first-page":"3703","DOI":"10.1074\/jbc.M113.538264","volume":"289","author":"E Workman","year":"2014","unstructured":"Workman E, Veith A, Battle DJ (2014) U1A Regulates 3\u2019 processing of the survival motor neuron mRNA. J Biol Chem 289(6):3703\u20133712. https:\/\/doi.org\/10.1074\/jbc.M113.538264","journal-title":"J Biol Chem"},{"key":"669_CR173","doi-asserted-by":"publisher","first-page":"1","DOI":"10.4061\/2011\/137459","volume":"2011","author":"W Xie","year":"2011","unstructured":"Xie W, Denman RB (2011) Protein methylation and stress granules: posttranslational remodeler or innocent bystander? Molecular Biology International 2011:1\u201314. https:\/\/doi.org\/10.4061\/2011\/137459","journal-title":"Molecular Biology International"},{"issue":"11","key":"669_CR174","doi-asserted-by":"publisher","first-page":"2051","DOI":"10.1038\/s41388-021-01693-w","volume":"40","author":"Y Xing","year":"2021","unstructured":"Xing Y et al (2021) Downregulation of NUDT21 contributes to cervical cancer progression through alternative polyadenylation. Oncogene 40(11):2051\u20132064. https:\/\/doi.org\/10.1038\/s41388-021-01693-w","journal-title":"Oncogene"},{"key":"669_CR175","doi-asserted-by":"publisher","unstructured":"Xu\u00a0Y et al\u00a0(2019) Post-translational modification control of RNA-binding protein hnRNPK function. Open Biol 9(3). https:\/\/doi.org\/10.1098\/rsob.180239","DOI":"10.1098\/rsob.180239"},{"issue":"32","key":"669_CR176","doi-asserted-by":"publisher","first-page":"10851","DOI":"10.1523\/jneurosci.1630-10.2010","volume":"30","author":"YF Xu","year":"2010","unstructured":"Xu YF et al (2010) Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice. J Neurosci 30(32):10851\u201310859. https:\/\/doi.org\/10.1523\/jneurosci.1630-10.2010","journal-title":"J Neurosci"},{"issue":"9","key":"669_CR177","doi-asserted-by":"publisher","first-page":"2017","DOI":"10.1038\/s41380-018-0240-0","volume":"25","author":"YM Yang","year":"2018","unstructured":"Yang YM et al (2018) Identification of a molecular locus for normalizing dysregulated GABA release from interneurons in the Fragile X brain. Mol Psychiatry 25(9):2017\u20132035. https:\/\/doi.org\/10.1038\/s41380-018-0240-0","journal-title":"Mol Psychiatry"},{"issue":"22","key":"669_CR178","doi-asserted-by":"publisher","first-page":"13254","DOI":"10.1073\/pnas.95.22.13254","volume":"95","author":"YYL Yang","year":"1998","unstructured":"Yang YYL, Yin GL, Darnell RB (1998) The neuronal RNA-binding protein Nova-2 is implicated as the autoantigen targeted in POMA patients with dementia. Proc Natl Acad Sci USA 95(22):13254\u201313259. https:\/\/doi.org\/10.1073\/pnas.95.22.13254","journal-title":"Proc Natl Acad Sci USA"},{"issue":"2","key":"669_CR179","doi-asserted-by":"publisher","first-page":"193","DOI":"10.1261\/rna.069237.118","volume":"25","author":"BA Yee","year":"2019","unstructured":"Yee BA, Pratt GA, Graveley BR, van Nostrand EL, Yeo GW (2019) RBP-Maps enables robust generation of splicing regulatory maps. RNA 25(2):193\u2013204. https:\/\/doi.org\/10.1261\/rna.069237.118","journal-title":"RNA"},{"issue":"3","key":"669_CR180","first-page":"2802","volume":"8","author":"Y Yu","year":"2015","unstructured":"Yu Y, Li Y, Zhang Y (2015) Screening of APP interaction proteins by DUALmembrane yeast two-hybrid system. Int J Clin Exp Pathol 8(3):2802\u20132808","journal-title":"Int J Clin Exp Pathol"},{"issue":"13","key":"669_CR181","doi-asserted-by":"publisher","first-page":"2442","DOI":"10.7150\/ijbs.46751","volume":"16","author":"X Zheng","year":"2020","unstructured":"Zheng X et al (2020) Serine\/arginine-rich splicing factors: The bridge linking alternative splicing and cancer. Int J Biol Sci 16(13):2442\u20132453. https:\/\/doi.org\/10.7150\/ijbs.46751","journal-title":"Int J Biol Sci"},{"issue":"5","key":"669_CR182","doi-asserted-by":"publisher","first-page":"1863","DOI":"10.3892\/or.2020.7769","volume":"44","author":"YI Zhong","year":"2020","unstructured":"Zhong YI, Hu Z, Wu J, Dai FAN, Lee F, Xu Y (2020) STAU1 selectively regulates the expression of inflammatory and immune response genes and alternative splicing of the nerve growth factor receptor signaling pathway. Oncol Rep 44(5):1863\u20131874. https:\/\/doi.org\/10.3892\/or.2020.7769","journal-title":"Oncol Rep"},{"issue":"6","key":"669_CR183","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1002\/brb3.1625","volume":"10","author":"B Zhou","year":"2020","unstructured":"Zhou B et al (2020) FUS P525L mutation causing amyotrophic lateral sclerosis and movement disorders. Brain and Behavior 10(6):1\u20139. https:\/\/doi.org\/10.1002\/brb3.1625","journal-title":"Brain and Behavior"},{"issue":"4","key":"669_CR184","doi-asserted-by":"publisher","first-page":"432","DOI":"10.1007\/s11427-014-4647-9","volume":"57","author":"HL Zhou","year":"2014","unstructured":"Zhou HL, Mangelsdorf M, Liu JH, Zhu L, Wu JY (2014) RNA-binding proteins in neurological diseases. Science China Life Sciences 57(4):432\u2013444. https:\/\/doi.org\/10.1007\/s11427-014-4647-9","journal-title":"Science China Life Sciences"},{"issue":"4","key":"669_CR185","doi-asserted-by":"publisher","first-page":"2203","DOI":"10.1074\/jbc.M609349200","volume":"282","author":"H Zhu","year":"2007","unstructured":"Zhu H, Zhou HL, Hasman RA, Lou H (2007) Hu proteins regulate polyadenylation by blocking sites containing U-rich sequences. J Biol Chem 282(4):2203\u20132210. https:\/\/doi.org\/10.1074\/jbc.M609349200","journal-title":"J Biol Chem"}],"container-title":["Neurotoxicity Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s12640-023-00669-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s12640-023-00669-w\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s12640-023-00669-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,28]],"date-time":"2023-11-28T13:25:07Z","timestamp":1701177907000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s12640-023-00669-w"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,9,30]]},"references-count":185,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2023,12]]}},"alternative-id":["669"],"URL":"https:\/\/doi.org\/10.1007\/s12640-023-00669-w","relation":{},"ISSN":["1029-8428","1476-3524"],"issn-type":[{"value":"1029-8428","type":"print"},{"value":"1476-3524","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,9,30]]},"assertion":[{"value":"15 March 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"15 March 2023","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"19 September 2023","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"30 September 2023","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare no competing interests.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing Interests"}},{"value":"The authors declare that they have no conflict of interest.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of Interest"}}]}}