Balancing RNA processing and innate immune response: Possible roles for SMN condensates in snRNP biogenesis
Biomolecular condensates like U-bodies are specialized cellular structures formed through multivalent interactions among intrinsically disordered regions. U-bodies sequester small nuclear ribonucleoprotein complexes (snRNPs) in the cytoplasm, and their formation in mammalian cells depends on stress...
Saved in:
| Published in | Biochimica et biophysica acta. General subjects Vol. 1869; no. 3; p. 130764 |
|---|---|
| Main Authors | , |
| Format | Journal Article |
| Language | English |
| Published |
Netherlands
Elsevier B.V
01.03.2025
|
| Subjects | |
| Online Access | Get full text |
| ISSN | 0304-4165 1872-8006 1872-8006 |
| DOI | 10.1016/j.bbagen.2025.130764 |
Cover
Loading…
| Abstract | Biomolecular condensates like U-bodies are specialized cellular structures formed through multivalent interactions among intrinsically disordered regions. U-bodies sequester small nuclear ribonucleoprotein complexes (snRNPs) in the cytoplasm, and their formation in mammalian cells depends on stress conditions. Because of their location adjacent to P-bodies, U-bodies have been considered potential sites for snRNP storage or turnover. SMN, a chaperone for snRNP biogenesis, forms condensates through its Tudor domain. In fly models, defects in SMN trigger innate immune responses similar to those observed with excess cytoplasmic snRNA during viral infection in mammalian cells. Additionally, spinal muscular atrophy (SMA), caused by SMN deficiency, is associated with inflammation. Therefore, SMN may help prevent innate immune aberrant activation due to defective snRNP biogenesis by forming U-bodies to sequester these molecules. Further studies on U-body functions may provide therapeutic insights for diseases related to RNA metabolism.
•Excessive snRNA or defective snRNA triggers activation of innate immune signaling.•Processing-defective snRNA accumulates in U-bodies, cytoplasmic foci overlapping with P-bodies.•Mutant animals lacking functional SMN exhibit innate immune activation and show defects in U-body formation.•U-bodies may modulate the innate immune response. |
|---|---|
| AbstractList | Biomolecular condensates like U-bodies are specialized cellular structures formed through multivalent interactions among intrinsically disordered regions. U-bodies sequester small nuclear ribonucleoprotein complexes (snRNPs) in the cytoplasm, and their formation in mammalian cells depends on stress conditions. Because of their location adjacent to P-bodies, U-bodies have been considered potential sites for snRNP storage or turnover. SMN, a chaperone for snRNP biogenesis, forms condensates through its Tudor domain. In fly models, defects in SMN trigger innate immune responses similar to those observed with excess cytoplasmic snRNA during viral infection in mammalian cells. Additionally, spinal muscular atrophy (SMA), caused by SMN deficiency, is associated with inflammation. Therefore, SMN may help prevent innate immune aberrant activation due to defective snRNP biogenesis by forming U-bodies to sequester these molecules. Further studies on U-body functions may provide therapeutic insights for diseases related to RNA metabolism. Biomolecular condensates like U-bodies are specialized cellular structures formed through multivalent interactions among intrinsically disordered regions. U-bodies sequester small nuclear ribonucleoprotein complexes (snRNPs) in the cytoplasm, and their formation in mammalian cells depends on stress conditions. Because of their location adjacent to P-bodies, U-bodies have been considered potential sites for snRNP storage or turnover. SMN, a chaperone for snRNP biogenesis, forms condensates through its Tudor domain. In fly models, defects in SMN trigger innate immune responses similar to those observed with excess cytoplasmic snRNA during viral infection in mammalian cells. Additionally, spinal muscular atrophy (SMA), caused by SMN deficiency, is associated with inflammation. Therefore, SMN may help prevent innate immune aberrant activation due to defective snRNP biogenesis by forming U-bodies to sequester these molecules. Further studies on U-body functions may provide therapeutic insights for diseases related to RNA metabolism.Biomolecular condensates like U-bodies are specialized cellular structures formed through multivalent interactions among intrinsically disordered regions. U-bodies sequester small nuclear ribonucleoprotein complexes (snRNPs) in the cytoplasm, and their formation in mammalian cells depends on stress conditions. Because of their location adjacent to P-bodies, U-bodies have been considered potential sites for snRNP storage or turnover. SMN, a chaperone for snRNP biogenesis, forms condensates through its Tudor domain. In fly models, defects in SMN trigger innate immune responses similar to those observed with excess cytoplasmic snRNA during viral infection in mammalian cells. Additionally, spinal muscular atrophy (SMA), caused by SMN deficiency, is associated with inflammation. Therefore, SMN may help prevent innate immune aberrant activation due to defective snRNP biogenesis by forming U-bodies to sequester these molecules. Further studies on U-body functions may provide therapeutic insights for diseases related to RNA metabolism. Biomolecular condensates like U-bodies are specialized cellular structures formed through multivalent interactions among intrinsically disordered regions. U-bodies sequester small nuclear ribonucleoprotein complexes (snRNPs) in the cytoplasm, and their formation in mammalian cells depends on stress conditions. Because of their location adjacent to P-bodies, U-bodies have been considered potential sites for snRNP storage or turnover. SMN, a chaperone for snRNP biogenesis, forms condensates through its Tudor domain. In fly models, defects in SMN trigger innate immune responses similar to those observed with excess cytoplasmic snRNA during viral infection in mammalian cells. Additionally, spinal muscular atrophy (SMA), caused by SMN deficiency, is associated with inflammation. Therefore, SMN may help prevent innate immune aberrant activation due to defective snRNP biogenesis by forming U-bodies to sequester these molecules. Further studies on U-body functions may provide therapeutic insights for diseases related to RNA metabolism. •Excessive snRNA or defective snRNA triggers activation of innate immune signaling.•Processing-defective snRNA accumulates in U-bodies, cytoplasmic foci overlapping with P-bodies.•Mutant animals lacking functional SMN exhibit innate immune activation and show defects in U-body formation.•U-bodies may modulate the innate immune response. |
| ArticleNumber | 130764 |
| Author | Maita, Hiroshi Nakagawa, Shinichi |
| Author_xml | – sequence: 1 givenname: Hiroshi surname: Maita fullname: Maita, Hiroshi email: maimai@pharm.hokudai.ac.jp organization: Graduate School of Life Science, Hokkaido University, Sapporo 060-0812, Japan – sequence: 2 givenname: Shinichi surname: Nakagawa fullname: Nakagawa, Shinichi organization: Graduate School of Life Science, Hokkaido University, Sapporo 060-0812, Japan |
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/39826814$$D View this record in MEDLINE/PubMed |
| BookMark | eNqNkc1O3DAUha0KVAboG1TISzaZOv5L0gUSRYUiwYCm7dpynBvkaWIPvjNIfft6lClLhDeWre9c3XPOMTkIMQAhn0s2L1mpv6zmbWufIMw542peClZp-YHMyrriRc2YPiAzJpgsZKnVETlGXLF8VKM-kiPR1FzXpZyRP9_sYIPz4YkuF5d0naIDxN3Tho76EOwGqB_HbQCaANcxIHyljzEz7ZC_4gBI-5joz_sFdTF0EDBLMEsphuXikbY-5i0BPZ6Sw94OCJ_29wn5ff3919WP4u7h5vbq8q5wQspNUXUSqqbVvLeq6StnWS2zIdbW4KxzvJFMW9tr1WqpJe9qLSpXSS45yK4RWpyQ82ludvO8BdyY0aODIRuFuEUjciJZ1KjmHaiqlKg136Fne3TbjtCZdfKjTX_N_ywzICfApRxPgv4VKZnZVWZWZqrM7CozU2VZdjHJIEfy4iEZdB6Cg84ncBvTRf_2gH8VRJ88 |
| Cites_doi | 10.3390/genes9110531 10.1038/nrm3185 10.1002/wrna.1523 10.1038/s41576-018-0073-3 10.1016/j.molcel.2012.12.009 10.1016/j.bbamcr.2021.119102 10.1002/wrna.1589 10.1002/wrna.87 10.1093/nar/gkab1061 10.1016/j.clineuro.2024.108462 10.1016/j.ydbio.2009.05.553 10.1093/nar/gkaa301 10.1002/bies.201000088 10.1101/cshperspect.a003707 10.1093/nar/gkt1271 10.1016/j.devcel.2024.06.017 10.1016/j.celrep.2016.08.047 10.15252/embj.2022111870 10.1093/nar/gkv157 10.1261/rna.057208.116 10.1242/jcs.261834 10.1038/s43856-023-00256-2 10.1073/pnas.0902020106 10.1093/hmg/8.5.775 10.1186/s12915-024-01888-z 10.1515/hsz-2018-0205 10.1016/j.sbi.2020.09.004 10.1016/j.cell.2011.06.043 10.18632/oncotarget.8420 10.3389/fgene.2021.652129 10.3390/biom5020528 10.1016/j.celrep.2017.05.056 10.1016/j.molcel.2015.01.013 10.1093/hmg/7.8.1269 10.1016/j.immuni.2020.03.017 10.1080/15476286.2016.1236168 10.1016/j.mib.2017.07.004 10.1002/pro.4466 10.1016/j.celrep.2019.04.029 10.1016/j.str.2012.08.024 10.1016/S0092-8674(00)80829-6 10.1038/nrn2670 10.1073/pnas.1412614111 10.1038/nature04531 10.1093/nar/gkab508 10.1523/JNEUROSCI.2208-10.2010 10.1073/pnas.0704977104 10.1002/cac2.12131 10.1101/gad.336891.120 10.1146/annurev-genet-120417-031441 10.1128/MCB.21.24.8289-8300.2001 10.1074/jbc.M115.667279 10.1080/15476286.2016.1249091 10.1016/j.cell.2018.07.023 10.1038/ncomms7171 10.1146/annurev-biochem-091719-064225 10.1016/j.celrep.2019.05.031 10.1128/MCB.01189-07 10.1093/molbev/msn175 10.3233/ISB-00142 10.1016/j.nbd.2016.06.015 10.15252/embr.202051342 10.1016/j.cell.2012.09.033 10.1371/journal.pgen.1008861 10.1371/journal.pone.0130974 10.1016/S1097-2765(02)00484-7 10.3390/ijms21113868 10.1073/pnas.2304302120 10.1126/science.1218469 10.1093/hmg/9.19.2869 10.1038/s41467-020-20191-3 10.1016/j.tcb.2004.03.010 10.1042/CBI20110147 10.1074/jbc.M115.659466 10.1261/rna.940708 10.1016/S0092-8674(00)81632-3 10.1016/j.yexcr.2010.05.001 10.3389/fncel.2022.982760 10.1016/j.celrep.2019.11.013 10.1007/s00441-011-1145-2 10.1016/j.isci.2023.107604 10.1016/j.cell.2011.08.047 10.1002/wrna.1645 10.1038/nrm3742 10.15252/embj.201490350 10.1083/jcb.201611108 10.1111/cns.12242 10.1146/annurev-biochem-013118-111024 10.1038/nsmb.2815 10.1016/j.cub.2006.04.037 10.1093/nar/gkaa743 10.1016/j.cell.2021.05.008 10.1038/s41586-022-05668-z 10.1016/j.yexcr.2011.09.001 10.1093/nar/gkab452 10.1002/1873-3468.13031 10.1038/s41594-021-00601-w |
| ContentType | Journal Article |
| Copyright | 2025 Elsevier B.V. Copyright © 2025 Elsevier B.V. All rights reserved. |
| Copyright_xml | – notice: 2025 Elsevier B.V. – notice: Copyright © 2025 Elsevier B.V. All rights reserved. |
| DBID | AAYXX CITATION CGR CUY CVF ECM EIF NPM 7X8 7S9 L.6 |
| DOI | 10.1016/j.bbagen.2025.130764 |
| DatabaseName | CrossRef Medline MEDLINE MEDLINE (Ovid) MEDLINE MEDLINE PubMed MEDLINE - Academic AGRICOLA AGRICOLA - Academic |
| DatabaseTitle | CrossRef MEDLINE Medline Complete MEDLINE with Full Text PubMed MEDLINE (Ovid) MEDLINE - Academic AGRICOLA AGRICOLA - Academic |
| DatabaseTitleList | MEDLINE MEDLINE - Academic AGRICOLA |
| Database_xml | – sequence: 1 dbid: NPM name: PubMed url: https://proxy.k.utb.cz/login?url=http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed sourceTypes: Index Database – sequence: 2 dbid: EIF name: MEDLINE url: https://proxy.k.utb.cz/login?url=https://www.webofscience.com/wos/medline/basic-search sourceTypes: Index Database |
| DeliveryMethod | fulltext_linktorsrc |
| Discipline | Chemistry Biology |
| EISSN | 1872-8006 |
| ExternalDocumentID | 39826814 10_1016_j_bbagen_2025_130764 S0304416525000091 |
| Genre | Research Support, Non-U.S. Gov't Journal Article Review |
| GroupedDBID | --- --K --M .~1 0R~ 1B1 1RT 1~. 1~5 23N 3O- 4.4 457 4G. 53G 5GY 5RE 5VS 7-5 71M 8P~ 9JM AACTN AAEDT AAEDW AAHBH AAIKJ AAKOC AALRI AAOAW AAQFI AAQXK AAXKI AAXUO ABEFU ABFNM ABGSF ABMAC ABUDA ABWVN ABXDB ACDAQ ACIUM ACRLP ACRPL ADBBV ADEZE ADMUD ADNMO ADUVX AEBSH AEHWI AEIPS AEKER AFJKZ AFKWA AFTJW AFXIZ AGHFR AGRDE AGUBO AGYEJ AHHHB AIEXJ AIKHN AITUG AJOXV AKRWK ALMA_UNASSIGNED_HOLDINGS AMFUW AMRAJ ANKPU ASPBG AVWKF AXJTR AZFZN BKOJK BLXMC CS3 EBS EFJIC EJD EO8 EO9 EP2 EP3 FDB FEDTE FGOYB FIRID FNPLU FYGXN G-2 G-Q GBLVA HLW HVGLF HZ~ IHE J1W KOM LX3 M41 MO0 N9A O-L O9- OAUVE OHT OZT P-8 P-9 PC. Q38 R2- ROL RPZ SBG SCC SDF SDG SDP SES SEW SPCBC SSU SSZ T5K UQL WH7 WUQ XJT XPP ~G- AATTM AAYWO AAYXX ACVFH ADCNI AEUPX AFPUW AGCQF AGQPQ AGRNS AIGII AIIUN AKBMS AKYEP APXCP BNPGV CITATION SSH CGR CUY CVF ECM EIF NPM 7X8 EFKBS 7S9 L.6 |
| ID | FETCH-LOGICAL-c344t-7d4e79b62fa59f7ca0848000b8ecacc29406aaf65b64642d8637c74242e4d9363 |
| IEDL.DBID | .~1 |
| ISSN | 0304-4165 1872-8006 |
| IngestDate | Wed Jul 02 04:35:42 EDT 2025 Tue Aug 12 08:02:12 EDT 2025 Fri May 30 11:00:00 EDT 2025 Tue Jul 01 04:54:28 EDT 2025 Sat Feb 15 15:52:32 EST 2025 |
| IsPeerReviewed | true |
| IsScholarly | true |
| Issue | 3 |
| Keywords | Tudor domain Symmetrical arginine dimethylation SMN Coilin Cajal body Intrinsically disordered region U-body |
| Language | English |
| License | Copyright © 2025 Elsevier B.V. All rights reserved. |
| LinkModel | DirectLink |
| MergedId | FETCHMERGED-LOGICAL-c344t-7d4e79b62fa59f7ca0848000b8ecacc29406aaf65b64642d8637c74242e4d9363 |
| Notes | ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 ObjectType-Review-3 content type line 23 |
| PMID | 39826814 |
| PQID | 3157538629 |
| PQPubID | 23479 |
| ParticipantIDs | proquest_miscellaneous_3165863959 proquest_miscellaneous_3157538629 pubmed_primary_39826814 crossref_primary_10_1016_j_bbagen_2025_130764 elsevier_sciencedirect_doi_10_1016_j_bbagen_2025_130764 |
| ProviderPackageCode | CITATION AAYXX |
| PublicationCentury | 2000 |
| PublicationDate | March 2025 2025-03-00 20250301 |
| PublicationDateYYYYMMDD | 2025-03-01 |
| PublicationDate_xml | – month: 03 year: 2025 text: March 2025 |
| PublicationDecade | 2020 |
| PublicationPlace | Netherlands |
| PublicationPlace_xml | – name: Netherlands |
| PublicationTitle | Biochimica et biophysica acta. General subjects |
| PublicationTitleAlternate | Biochim Biophys Acta Gen Subj |
| PublicationYear | 2025 |
| Publisher | Elsevier B.V |
| Publisher_xml | – name: Elsevier B.V |
| References | Cauchi, Sanchez-Pulido, Liu (bb0295) 2010; 316 Jaglarz, Kloc, Jankowska, Szymanska, Bilinski (bb0420) 2011; 344 Gonsalvez, Gonsalvez, Praveen, Praveen, Hicks, Hicks, Tian, Matera (bb0285) 2008; 14 Bonanno, Cavalcante, Salvi, Giagnorio, Malacarne, Cattaneo, Andreetta, Venerando, Pensato, Gellera, Zanin, Arnoldi, Dosi, Mantegazza, Masson, Maggi, Marcuzzo (bb0480) 2022; 16 Martin, Gupta, Ninan, Perry, Van Duyne (bb0330) 2012; 20 Schmitz, Cox, Esser, Voss, Sander, Löffler, Hillebrand, Erkelenz, Schaal, Kähne, Klinker, Zhang, Nagel-Steger, Willbold, Seggewiß, Schlütermann, Stork, Grimmler, Wesselborg, Peter (bb0210) 2021; 49 Paknia, Chari, Stark, Fischer (bb0145) 2016; 16 Roithová, Feketová, Vaňáčová, Staněk (bb0180) 2020; 48 Neuenkirchen, Englbrecht, Ohmer, Ziegenhals, Chari, Fischer (bb0155) 2015; 34 Saiz, Martinez-Salas (bb0460) 2021; 12 Courchaine, Barentine, Straube, Lee, Bewersdorf, Neugebauer (bb0365) 2021; 184 Wan, Bai, Zhan, Shi (bb0050) 2020; 89 Gonsalvez, Rajendra, Tian, Matera (bb0465) 2006; 16 Lee, Davies, Liu (bb0290) 2009; 332 Gupta, Martin, Sharp, Sarachan (bb0335) 2015; 290 Burghes, Beattie (bb0360) 2009; 10 Yin, Lopez-Gonzalez, Kunz, Gangopadhyay, Borufka, Gygi, Gao, Reed (bb0525) 2017; 19 Deguise, De Repentigny, McFall, Auclair, Sad, Kothary (bb0495) 2017; 26 Becker, Hirsch, Bender, Lingner, Salinas, Krebber (bb0085) 2019; 27 Pellizzoni, Kataoka, Charroux, Dreyfuss (bb0300) 1998; 95 Gao, Jing, Shang, Chen, Duan, Yu, Wang, Luo, Song, Chen, Chen, Zhang, Ding (bb0395) 2024 Zhang, So, Li, Yong, Glisovic, Wan, Dreyfuss (bb0160) 2011; 146 Zhang, Kerbl-Knapp, Rodriguez Colman, Meinitzer, Macher, Vujić, Fasching, Jany-Luig, Korbelius, Kuentzel, Mack, Akhmetshina, Pirchheim, Paar, Rinner, Hörl, Steyrer, Stelzl, Burgering, Eisenberg, Pertschy, Kratky, Madl (bb0430) 2021; 1 Cheng, Li, Fan, Zhao, Li, Lu, Zhu, Niu (bb0490) 2024; 244 Tsalikis, Tattoli, Ling, Sorbara, Croitoru, Philpott, Girardin (bb0035) 2015; 290 Borcherds, Bremer, Borgia, Mittag (bb0010) 2021; 67 Weiss, Diederichs, Read, Panjikar, Van Duyne, Matera, Fischer, Grimm (bb0310) 2016; 25 Nesterov, Ilyinsky, Uversky (bb0015) 2021; 1868 Scofield, Lynch (bb0165) 2008; 25 Lorson, Androphy (bb0345) 1998; 7 Van Treeck, Parker (bb0005) 2018; 174 Kitao, Segref, Kast, Wilm, Mattaj, Ohno (bb0120) 2008; 28 Chen, Nott, Jin, Pawson (bb0380) 2011; 12 Will, Lührmann (bb0045) 2011; 3 Cauchi (bb0530) 2014; 20 Ishizu, Kinoshita, Hirakata, Komatsuzaki, Siomi (bb0405) 2019; 27 Garcia, Wen, Praveen, Matera (bb0440) 2016; 22 Stepanov, Zhuravlev, Shender, Nushtaeva, Balakhonova, Mozhaeva, Kasakin, Koval, Lomzov, Pavlyukov, Malyants, Zhorov, Kabilova, Chernolovskaya, Govorun, Kuligina, Semenov, Richter (bb0270) 2018; 9 Buckingham, Liu (bb0040) 2011; 317 Martinez-Salas, Embarc-Buh, Francisco-Velilla (bb0125) 2020; 21 Bertrandy, Burlet, Clermont, Huber, Fondrat, Thierry-Mieg, Munnich, Lefebvre (bb0350) 1999; 8 Fica, Mefford, Piccirilli, Staley (bb0250) 2014; 21 Grimm, Chari, Pelz, Kuper, Kisker, Diederichs, Stark, Schindelin, Fischer (bb0150) 2013; 49 Wang, Barbash, Kelleher (bb0410) 2020; 16 Liu, Gall (bb0030) 2007; 104 Young, Man, Lorson, Le, Androphy, Burghes, Morris (bb0355) 2000; 9 Morais, Adachi, Yu (bb0095) 2021; 12 Cauchi (bb0280) 2011; 35 Ohazama, Fujimoto, Konda, Yokoyama, Nakagawa, Maita (bb0305) 2024; 137 Hirose, Ninomiya, Nakagawa, Yamazaki (bb0025) 2022 Czech, Munafò, Ciabrelli, Eastwood, Fabry, Kneuss, Hannon (bb0385) 2018; 52 Liu, Gack (bb0220) 2020; 53 Matera, Wang (bb0075) 2014; 15 Hu, Hou, Zhou, Wang, Shen, Mu, Su, Zhang (bb0375) 2023; 26 Chung, Shoji, Izumi, Tomari (bb0415) 2021; 22 Marcia, Pyle (bb0245) 2012; 151 Shi, Shi, Shen, Zhang, Cao (bb0425) 2021; 41 Haack, Toor (bb0235) 2020; 11 Vukojicic, Delestrée, Fletcher, Pagiazitis, Sankaranarayanan, Yednock, Barres, Mentis (bb0505) 2019; 29 Despic, Jaffrey (bb0170) 2023; 614 Guruharsha, Rual, Zhai, Mintseris, Vaidya, Vaidya, Beekman, Wong, Rhee, Cenaj, McKillip, Shah, Stapleton, Wan, Yu, Parsa, Carlson, Chen, Kapadia, VijayRaghavan, Gygi, Celniker, Obar, Artavanis-Tsakonas (bb0450) 2011; 147 Jiang, Zou, Zhang, Chen, Li, Wang, Deng, Wang, Chen (bb0195) 2018; 592 Valadkhan, Mohammadi, Jaladat, Geisler (bb0240) 2009; 106 Mouaikel, Verheggen, Bertrand, Tazi, Bordonné (bb0080) 2002; 9 Piñeiro, Fernández-Chamorro, Francisco-Velilla, Martínez-Salas (bb0455) 2015; 5 Wiedner, Giudice (bb0020) 2021; 28 Ozata, Gainetdinov, Zoch, O’Carroll, Zamore (bb0390) 2019; 20 Nuzzo, Russo, Errico, D’Amico, Tewelde, Valletta, Hassan, Tosi, Panicucci, Bruno, Bertini, Chambery, Pellizzoni, Usiello (bb0485) 2023; 3 Zhang, Vielle, Espinosa, Zhao (bb0255) 2019; 10 Wilkinson, Charenton, Nagai (bb0055) 2020; 89 Nott, Petsalaki, Farber, Jervis, Fussner, Plochowietz, Craggs, Bazett-Jones, Pawson, Forman-Kay, Baldwin (bb0400) 2015; 57 Garcia, Steiner, Raimer, Herring, Matera, Spring (bb0445) 2024; 22 Martin, Koonin (bb0230) 2006; 440 Corbet, Burke, Parker (bb0225) 2023; 42 Stejskalová, Staněk (bb0275) 2014; 127 Ma, Xiong, Lardelli, Lykke-Andersen (bb0105) 2024; 121 Alderson, Pritišanac, Kolarić, Moses, Forman-Kay (bb0325) 2023; 120 Ishikawa, Nobe, Izumikawa, Yoshikawa, Miyazawa, Terukina, Kurokawa, Taoka, Yamauchi, Nakayama, Isobe, Takahashi (bb0135) 2014; 42 Yu, Chi, Xia, Gangopadhyay, Yamazaki, Winkelbauer-Hurt, Yin, Eliasse, Adams, Shaw, Reed (bb0510) 2015; 43 Sun, Ling, Qiu, Albuquerque, Zhou, Tokunaga, Li, Qiu, Bui, Yeo, Huang, Eggan, Zhou, Fu, Lagier-Tourenne, Cleveland (bb0515) 2015; 6 Shukla, Parker (bb0175) 2014; 111 Ohno, Segref, Bachi, Wilm, Mattaj (bb0110) 2000; 101 Khairallah, Astroski, Custer, Androphy, Franklin, Lorson (bb0500) 2017; 26 Jutzi, Campagne, Schmidt, Reber, Mechtersheimer, Gypas, Schweingruber, Colombo, von Schroetter, Loughlin, Devoy, Hedlund, Zavolan, Allain, Ruepp (bb0520) 2020; 11 Varadi, Anyango, Deshpande, Nair, Natassia, Yordanova, Yuan, Stroe, Wood, Laydon, Žídek, Green, Tunyasuvunakool, Petersen, Jumper, Clancy, Green, Vora, Lutfi, Figurnov, Cowie, Hobbs, Kohli, Kleywegt, Birney, Hassabis, Velankar (bb0315) 2022; 50 Ranoa, Parekh, Pitroda, Huang, Darga, Wong, Huang, Andrade, Staley, Satoh, Akira, Weichselbaum, Khodarev (bb0260) 2016; 7 Wan, Feng, Guan, Sheng, Liu, Hua (bb0475) 2018; 27 Colin, Sourjik (bb0435) 2017; 39 McCloskey, Taniguchi, Shinmyozu, Ohno (bb0115) 2012; 335 Yong, Wan, Dreyfuss (bb0215) 2004; 14 Sakharkar, Chow, Kangueane (bb0060) 2004; 4 Raimer, Gray, Matera (bb0370) 2017; 14 Park, Maeno-Hikichi, Awano, Landmesser, Monani (bb0470) 2010; 30 Borg, Bordonne, Vassallo, Cauchi (bb0200) 2015; 10 Dou, Kalmykova, Pashkova, Oghbaie, Jiang, Molloy, Chait, Rout, Fenyö, Jensen, Altukhov, LaCava (bb0130) 2020; 48 Lardelli, Lykke-Andersen (bb0185) 2020; 34 Piovesan, Monzon, Tosatto (bb0320) 2022; 31 Friesen, Paushkin, Wyce, Massenet, Pesiridis, Van Duyne, Rappsilber, Mann, Dreyfuss (bb0140) 2001; 21 Meier (bb0090) 2017; 14 Cauchi (bb0065) 2010; 32 Fischer, Englbrecht, Chari (bb0070) 2011; 2 Gupta, Wen, Ninan, Raimer, Sharp, Spring, Sarachan, Johnson, Van Duyne, Matera (bb0340) 2021; 49 Zhang, Liu, Qiao, Li, Han, Sun, Ge, Zhu, Li, Yao, Zhang, Dai, Yan, Chen, Yin, Ma (bb0265) 2024; 43 Borg, Fenech Salerno, Vassallo, Bordonne, Cauchi (bb0205) 2016; 94 Prusty, Meduri, Prusty, Vanselow, Schlosser, Fischer (bb0190) 2017; 216 Bohnsack, Sloan (bb0100) 2018; 399 McCloskey (10.1016/j.bbagen.2025.130764_bb0115) 2012; 335 Gonsalvez (10.1016/j.bbagen.2025.130764_bb0285) 2008; 14 Borcherds (10.1016/j.bbagen.2025.130764_bb0010) 2021; 67 Hirose (10.1016/j.bbagen.2025.130764_bb0025) 2022 Sakharkar (10.1016/j.bbagen.2025.130764_bb0060) 2004; 4 Lorson (10.1016/j.bbagen.2025.130764_bb0345) 1998; 7 Zhang (10.1016/j.bbagen.2025.130764_bb0160) 2011; 146 Czech (10.1016/j.bbagen.2025.130764_bb0385) 2018; 52 Neuenkirchen (10.1016/j.bbagen.2025.130764_bb0155) 2015; 34 Nott (10.1016/j.bbagen.2025.130764_bb0400) 2015; 57 Raimer (10.1016/j.bbagen.2025.130764_bb0370) 2017; 14 Garcia (10.1016/j.bbagen.2025.130764_bb0445) 2024; 22 Bonanno (10.1016/j.bbagen.2025.130764_bb0480) 2022; 16 Gupta (10.1016/j.bbagen.2025.130764_bb0340) 2021; 49 Bohnsack (10.1016/j.bbagen.2025.130764_bb0100) 2018; 399 Colin (10.1016/j.bbagen.2025.130764_bb0435) 2017; 39 Cauchi (10.1016/j.bbagen.2025.130764_bb0295) 2010; 316 Chen (10.1016/j.bbagen.2025.130764_bb0380) 2011; 12 Matera (10.1016/j.bbagen.2025.130764_bb0075) 2014; 15 Scofield (10.1016/j.bbagen.2025.130764_bb0165) 2008; 25 Shi (10.1016/j.bbagen.2025.130764_bb0425) 2021; 41 Prusty (10.1016/j.bbagen.2025.130764_bb0190) 2017; 216 Corbet (10.1016/j.bbagen.2025.130764_bb0225) 2023; 42 Ozata (10.1016/j.bbagen.2025.130764_bb0390) 2019; 20 Vukojicic (10.1016/j.bbagen.2025.130764_bb0505) 2019; 29 Will (10.1016/j.bbagen.2025.130764_bb0045) 2011; 3 Borg (10.1016/j.bbagen.2025.130764_bb0205) 2016; 94 Morais (10.1016/j.bbagen.2025.130764_bb0095) 2021; 12 Cauchi (10.1016/j.bbagen.2025.130764_bb0065) 2010; 32 Martin (10.1016/j.bbagen.2025.130764_bb0230) 2006; 440 Hu (10.1016/j.bbagen.2025.130764_bb0375) 2023; 26 Khairallah (10.1016/j.bbagen.2025.130764_bb0500) 2017; 26 Alderson (10.1016/j.bbagen.2025.130764_bb0325) 2023; 120 Grimm (10.1016/j.bbagen.2025.130764_bb0150) 2013; 49 Marcia (10.1016/j.bbagen.2025.130764_bb0245) 2012; 151 Pellizzoni (10.1016/j.bbagen.2025.130764_bb0300) 1998; 95 Ranoa (10.1016/j.bbagen.2025.130764_bb0260) 2016; 7 Borg (10.1016/j.bbagen.2025.130764_bb0200) 2015; 10 Stepanov (10.1016/j.bbagen.2025.130764_bb0270) 2018; 9 Weiss (10.1016/j.bbagen.2025.130764_bb0310) 2016; 25 Ohazama (10.1016/j.bbagen.2025.130764_bb0305) 2024; 137 Sun (10.1016/j.bbagen.2025.130764_bb0515) 2015; 6 Tsalikis (10.1016/j.bbagen.2025.130764_bb0035) 2015; 290 Lardelli (10.1016/j.bbagen.2025.130764_bb0185) 2020; 34 Guruharsha (10.1016/j.bbagen.2025.130764_bb0450) 2011; 147 Schmitz (10.1016/j.bbagen.2025.130764_bb0210) 2021; 49 Chung (10.1016/j.bbagen.2025.130764_bb0415) 2021; 22 Courchaine (10.1016/j.bbagen.2025.130764_bb0365) 2021; 184 Park (10.1016/j.bbagen.2025.130764_bb0470) 2010; 30 Mouaikel (10.1016/j.bbagen.2025.130764_bb0080) 2002; 9 Ishikawa (10.1016/j.bbagen.2025.130764_bb0135) 2014; 42 Young (10.1016/j.bbagen.2025.130764_bb0355) 2000; 9 Kitao (10.1016/j.bbagen.2025.130764_bb0120) 2008; 28 Yong (10.1016/j.bbagen.2025.130764_bb0215) 2004; 14 Valadkhan (10.1016/j.bbagen.2025.130764_bb0240) 2009; 106 Garcia (10.1016/j.bbagen.2025.130764_bb0440) 2016; 22 Jiang (10.1016/j.bbagen.2025.130764_bb0195) 2018; 592 Nesterov (10.1016/j.bbagen.2025.130764_bb0015) 2021; 1868 Liu (10.1016/j.bbagen.2025.130764_bb0220) 2020; 53 Gupta (10.1016/j.bbagen.2025.130764_bb0335) 2015; 290 Roithová (10.1016/j.bbagen.2025.130764_bb0180) 2020; 48 Cheng (10.1016/j.bbagen.2025.130764_bb0490) 2024; 244 Stejskalová (10.1016/j.bbagen.2025.130764_bb0275) 2014; 127 Saiz (10.1016/j.bbagen.2025.130764_bb0460) 2021; 12 Bertrandy (10.1016/j.bbagen.2025.130764_bb0350) 1999; 8 Wan (10.1016/j.bbagen.2025.130764_bb0050) 2020; 89 Nuzzo (10.1016/j.bbagen.2025.130764_bb0485) 2023; 3 Yu (10.1016/j.bbagen.2025.130764_bb0510) 2015; 43 Wiedner (10.1016/j.bbagen.2025.130764_bb0020) 2021; 28 Fica (10.1016/j.bbagen.2025.130764_bb0250) 2014; 21 Wang (10.1016/j.bbagen.2025.130764_bb0410) 2020; 16 Deguise (10.1016/j.bbagen.2025.130764_bb0495) 2017; 26 Ohno (10.1016/j.bbagen.2025.130764_bb0110) 2000; 101 Martin (10.1016/j.bbagen.2025.130764_bb0330) 2012; 20 Martinez-Salas (10.1016/j.bbagen.2025.130764_bb0125) 2020; 21 Cauchi (10.1016/j.bbagen.2025.130764_bb0530) 2014; 20 Piovesan (10.1016/j.bbagen.2025.130764_bb0320) 2022; 31 Gonsalvez (10.1016/j.bbagen.2025.130764_bb0465) 2006; 16 Despic (10.1016/j.bbagen.2025.130764_bb0170) 2023; 614 Burghes (10.1016/j.bbagen.2025.130764_bb0360) 2009; 10 Lee (10.1016/j.bbagen.2025.130764_bb0290) 2009; 332 Paknia (10.1016/j.bbagen.2025.130764_bb0145) 2016; 16 Varadi (10.1016/j.bbagen.2025.130764_bb0315) 2022; 50 Wan (10.1016/j.bbagen.2025.130764_bb0475) 2018; 27 Becker (10.1016/j.bbagen.2025.130764_bb0085) 2019; 27 Meier (10.1016/j.bbagen.2025.130764_bb0090) 2017; 14 Wilkinson (10.1016/j.bbagen.2025.130764_bb0055) 2020; 89 Gao (10.1016/j.bbagen.2025.130764_bb0395) 2024 Ishizu (10.1016/j.bbagen.2025.130764_bb0405) 2019; 27 Jutzi (10.1016/j.bbagen.2025.130764_bb0520) 2020; 11 Zhang (10.1016/j.bbagen.2025.130764_bb0430) 2021; 1 Friesen (10.1016/j.bbagen.2025.130764_bb0140) 2001; 21 Fischer (10.1016/j.bbagen.2025.130764_bb0070) 2011; 2 Zhang (10.1016/j.bbagen.2025.130764_bb0255) 2019; 10 Buckingham (10.1016/j.bbagen.2025.130764_bb0040) 2011; 317 Piñeiro (10.1016/j.bbagen.2025.130764_bb0455) 2015; 5 Cauchi (10.1016/j.bbagen.2025.130764_bb0280) 2011; 35 Shukla (10.1016/j.bbagen.2025.130764_bb0175) 2014; 111 Yin (10.1016/j.bbagen.2025.130764_bb0525) 2017; 19 Zhang (10.1016/j.bbagen.2025.130764_bb0265) 2024; 43 Haack (10.1016/j.bbagen.2025.130764_bb0235) 2020; 11 Jaglarz (10.1016/j.bbagen.2025.130764_bb0420) 2011; 344 Ma (10.1016/j.bbagen.2025.130764_bb0105) 2024; 121 Liu (10.1016/j.bbagen.2025.130764_bb0030) 2007; 104 Van Treeck (10.1016/j.bbagen.2025.130764_bb0005) 2018; 174 Dou (10.1016/j.bbagen.2025.130764_bb0130) 2020; 48 |
| References_xml | – volume: 50 start-page: D439 year: 2022 end-page: D444 ident: bb0315 article-title: AlphaFold protein structure database: massively expanding the structural coverage of protein-sequence space with high-accuracy models publication-title: Nucleic Acids Res. – volume: 20 start-page: 89 year: 2019 end-page: 108 ident: bb0390 article-title: PIWI-interacting RNAs: small RNAs with big functions publication-title: Nat. Rev. Genet. – volume: 89 start-page: 359 year: 2020 end-page: 388 ident: bb0055 article-title: RNA splicing by the spliceosome publication-title: Annu. Rev. Biochem. – volume: 14 start-page: 878 year: 2008 end-page: 887 ident: bb0285 article-title: Sm protein methylation is dispensable for snRNP assembly in publication-title: RNA – volume: 26 start-page: 932 year: 2017 end-page: 941 ident: bb0500 article-title: SMN deficiency negatively impacts red pulp macrophages and spleen development in mouse models of spinal muscular atrophy publication-title: Hum. Mol. Genet. – volume: 12 year: 2021 ident: bb0095 article-title: Spliceosomal snRNA Epitranscriptomics publication-title: Front. Genet. – volume: 5 start-page: 528 year: 2015 end-page: 544 ident: bb0455 article-title: Gemin5: a multitasking RNA-binding protein involved in translation control publication-title: Biomolecules – volume: 29 start-page: 3087 year: 2019 end-page: 3100 ident: bb0505 article-title: The classical complement pathway mediates microglia-dependent remodeling of spinal motor circuits during development and in SMA publication-title: Cell Rep. – volume: 3 start-page: 28 year: 2023 ident: bb0485 article-title: Nusinersen mitigates neuroinflammation in severe spinal muscular atrophy patients publication-title: Commun. Med. (Lond.) – volume: 53 start-page: 26 year: 2020 end-page: 42 ident: bb0220 article-title: Distinct and orchestrated functions of RNA sensors in innate immunity publication-title: Immunity – volume: 28 start-page: 465 year: 2021 end-page: 473 ident: bb0020 article-title: It’s not just a phase: function and characteristics of RNA-binding proteins in phase separation publication-title: Nat. Struct. Mol. Biol. – volume: 2 start-page: 718 year: 2011 end-page: 731 ident: bb0070 article-title: Biogenesis of spliceosomal small nuclear ribonucleoproteins: biogenesis of spliceosomal snRNPs publication-title: Wiley Interdiscip. Rev. RNA – volume: 8 start-page: 775 year: 1999 end-page: 782 ident: bb0350 article-title: The RNA-binding properties of SMN: deletion analysis of the zebrafish orthologue defines domains conserved in evolution publication-title: Hum. Mol. Genet. – volume: 10 year: 2019 ident: bb0255 article-title: RNAs in the spliceosome: insight from cryoEM structures publication-title: Wiley Interdiscip. Rev. RNA – volume: 151 start-page: 497 year: 2012 end-page: 507 ident: bb0245 article-title: Visualizing group II intron catalysis through the stages of splicing publication-title: Cell – volume: 244 year: 2024 ident: bb0490 article-title: Cytokines in cerebrospinal fluid as a prognostic predictor after treatment of nusinersen in SMA patients publication-title: Clin. Neurol. Neurosurg. – volume: 6 start-page: 6171 year: 2015 ident: bb0515 article-title: ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP publication-title: Nat. Commun. – volume: 16 start-page: 3103 year: 2016 end-page: 3112 ident: bb0145 article-title: The ribosome cooperates with the assembly chaperone pICln to initiate formation of snRNPs publication-title: Cell Rep. – volume: 335 start-page: 1643 year: 2012 end-page: 1646 ident: bb0115 article-title: hnRNP C tetramer measures RNA length to classify RNA polymerase II transcripts for export publication-title: Science – volume: 43 start-page: 3208 year: 2015 end-page: 3218 ident: bb0510 article-title: U1 snRNP is mislocalized in ALS patient fibroblasts bearing NLS mutations in FUS and is required for motor neuron outgrowth in zebrafish publication-title: Nucleic Acids Res. – volume: 10 year: 2015 ident: bb0200 article-title: Genetic interactions between the members of the SMN-Gemins complex in Drosophila publication-title: PLoS One – volume: 21 start-page: 464 year: 2014 end-page: 471 ident: bb0250 article-title: Evidence for a group II intron-like catalytic triplex in the spliceosome publication-title: Nat. Struct. Mol. Biol. – volume: 25 start-page: 2255 year: 2008 end-page: 2267 ident: bb0165 article-title: Evolutionary diversification of the Sm family of RNA-associated proteins publication-title: Mol. Biol. Evol. – volume: 216 start-page: 2391 year: 2017 end-page: 2407 ident: bb0190 article-title: Impaired spliceosomal UsnRNP assembly leads to Sm mRNA down-regulation and Sm protein degradation publication-title: J. Cell Biol. – volume: 22 start-page: 1215 year: 2016 end-page: 1227 ident: bb0440 article-title: Transcriptomic comparison of Drosophila snRNP biogenesis mutants reveals mutant-specific changes in pre-mRNA processing: implications for spinal muscular atrophy publication-title: RNA – volume: 127 start-page: 3909 year: 2014 end-page: 3915 ident: bb0275 article-title: The splicing factor U1-70K interacts with the SMN complex and is required for nuclear gem integrity publication-title: J. Cell Sci. – volume: 95 start-page: 615 year: 1998 end-page: 624 ident: bb0300 article-title: A novel function for SMN, the spinal muscular atrophy disease gene product, in pre-mRNA splicing publication-title: Cell – volume: 1868 year: 2021 ident: bb0015 article-title: Liquid-liquid phase separation as a common organizing principle of intracellular space and biomembranes providing dynamic adaptive responses publication-title: Biochim. Biophys. Acta, Mol. Cell Res. – volume: 614 start-page: 358 year: 2023 end-page: 366 ident: bb0170 article-title: mRNA ageing shapes the Cap2 methylome in mammalian mRNA publication-title: Nature – volume: 49 start-page: 692 year: 2013 end-page: 703 ident: bb0150 article-title: Structural basis of assembly chaperone- mediated snRNP formation publication-title: Mol. Cell – volume: 14 start-page: 693 year: 2017 end-page: 700 ident: bb0090 article-title: RNA modification in Cajal bodies publication-title: RNA Biol. – volume: 31 year: 2022 ident: bb0320 article-title: Intrinsic protein disorder and conditional folding in AlphaFoldDB publication-title: Protein Sci. – volume: 290 start-page: 20185 year: 2015 end-page: 20199 ident: bb0335 article-title: Oligomeric properties of survival motor neuron·Gemin2 complexes publication-title: J. Biol.Chem. – volume: 26 start-page: 801 year: 2017 end-page: 819 ident: bb0495 article-title: Immune dysregulation may contribute to disease pathogenesis in spinal muscular atrophy mice publication-title: Hum. Mol. Genet. – volume: 316 start-page: 2354 year: 2010 end-page: 2364 ident: bb0295 article-title: Drosophila SMN complex proteins Gemin2, Gemin3, and Gemin5 are components of U bodies publication-title: Exp. Cell Res. – volume: 11 start-page: 6341 year: 2020 ident: bb0520 article-title: Aberrant interaction of FUS with the U1 snRNA provides a molecular mechanism of FUS induced amyotrophic lateral sclerosis publication-title: Nat. Commun. – volume: 7 start-page: 26496 year: 2016 end-page: 26515 ident: bb0260 article-title: Cancer therapies activate RIG-I-like receptor pathway through endogenous non-coding RNAs publication-title: Oncotarget – volume: 7 start-page: 1269 year: 1998 end-page: 1275 ident: bb0345 article-title: The domain encoded by exon 2 of the survival motor neuron protein mediates nucleic acid binding publication-title: Hum. Mol. Genet. – volume: 9 start-page: 891 year: 2002 end-page: 901 ident: bb0080 article-title: Hypermethylation of the cap structure of both yeast snRNAs and snoRNAs requires a conserved methyltransferase that is localized to the nucleolus publication-title: Mol. Cell – volume: 399 start-page: 1265 year: 2018 end-page: 1276 ident: bb0100 article-title: Modifications in small nuclear RNAs and their roles in spliceosome assembly and function publication-title: Biol. Chem. – volume: 34 start-page: 1925 year: 2015 end-page: 1941 ident: bb0155 article-title: Reconstitution of the human U snRNP assembly machinery reveals stepwise Sm protein organization publication-title: EMBO J. – volume: 332 start-page: 142 year: 2009 end-page: 155 ident: bb0290 article-title: The spinal muscular atrophy protein SMN affects Drosophila germline nuclear organization through the U body–P body pathway publication-title: Dev. Biol. – volume: 120 year: 2023 ident: bb0325 article-title: Systematic identification of conditionally folded intrinsically disordered regions by AlphaFold2 publication-title: Proc. Natl. Acad. Sci. USA – volume: 174 start-page: 791 year: 2018 end-page: 802 ident: bb0005 article-title: Emerging roles for intermolecular RNA-RNA interactions in RNP assemblies publication-title: Cell – volume: 3 year: 2011 ident: bb0045 article-title: Spliceosome structure and function publication-title: Cold Spring Harb. Perspect. Biol. – volume: 94 start-page: 245 year: 2016 end-page: 258 ident: bb0205 article-title: Disruption of snRNP biogenesis factors Tgs1 and pICln induces phenotypes that mirror aspects of SMN-Gemins complex perturbation in Drosophila, providing new insights into spinal muscular atrophy publication-title: Neurobiol. Dis. – volume: 27 start-page: 3199 year: 2019 end-page: 3214.e3 ident: bb0085 article-title: Nuclear pre-snRNA export is an essential quality assurance mechanism for functional spliceosomes publication-title: Cell Rep. – volume: 12 year: 2021 ident: bb0460 article-title: Uncovering targets of the leader protease: linking RNA-mediated pathways and antiviral defense publication-title: Wiley Interdiscip. Rev. RNA – volume: 146 start-page: 384 year: 2011 end-page: 395 ident: bb0160 article-title: Structure of a key intermediate of the SMN complex reveals Gemin2’s crucial function in snRNP assembly publication-title: Cell – volume: 20 start-page: 574 year: 2014 end-page: 581 ident: bb0530 article-title: Gem depletion: amyotrophic lateral sclerosis and spinal muscular atrophy crossover publication-title: CNS Neurosci. Ther. – volume: 89 start-page: 333 year: 2020 end-page: 358 ident: bb0050 article-title: How is precursor messenger RNA spliced by the spliceosome? publication-title: Annu. Rev. Biochem. – volume: 57 start-page: 936 year: 2015 end-page: 947 ident: bb0400 article-title: Phase transition of a disordered nuage protein generates environmentally responsive membraneless organelles publication-title: Mol. Cell – volume: 1 year: 2021 ident: bb0430 article-title: Global analysis of protein arginine methylation publication-title: Cell Rep. Methods – volume: 43 year: 2024 ident: bb0265 article-title: Housekeeping U1 snRNA facilitates antiviral innate immunity by promoting TRIM25-mediated RIG-I activation publication-title: Cell Rep. – volume: 9 start-page: 531 year: 2018 ident: bb0270 article-title: Nucleotide modifications decrease innate immune response induced by synthetic analogs of snRNAs and snoRNAs publication-title: Genes (Basel) – volume: 16 year: 2020 ident: bb0410 article-title: Adaptive evolution among cytoplasmic piRNA proteins leads to decreased genomic auto-immunity publication-title: PLoS Genet. – volume: 111 start-page: E3277 year: 2014 end-page: E3286 ident: bb0175 article-title: Quality control of assembly-defective U1 snRNAs by decapping and 5′-to-3′ exonucleolytic digestion publication-title: Proc. Natl. Acad. Sci. USA – volume: 35 start-page: 1233 year: 2011 end-page: 1238 ident: bb0280 article-title: Gem formation upon constitutive Gemin3 overexpression in Drosophila publication-title: Cell Biol. Int. – year: 2022 ident: bb0025 article-title: A guide to membraneless organelles and their various roles in gene regulation publication-title: Nat. Rev. Mol. Cell Biol. – volume: 22 start-page: 94 year: 2024 ident: bb0445 article-title: Dysregulation of innate immune signaling in animal models of spinal muscular atrophy publication-title: BMC Biol. – volume: 32 start-page: 1077 year: 2010 end-page: 1089 ident: bb0065 article-title: SMN and Gemins: “we are family” … or are we?: insights into the partnership between Gemins and the spinal muscular atrophy disease protein SMN: insights into the partnership between Gemins and the spinal muscular atrophy disease protein SMN publication-title: Bioessays – volume: 104 start-page: 11655 year: 2007 end-page: 11659 ident: bb0030 article-title: U bodies are cytoplasmic structures that contain uridine-rich small nuclear ribonucleoproteins and associate with P bodies publication-title: Proc. Natl. Acad. Sci. USA – volume: 25 start-page: 4717 year: 2016 end-page: 4725 ident: bb0310 article-title: A critical examination of the recently reported crystal structures of the human SMN protein publication-title: Hum. Mol. Genet. – volume: 16 start-page: 1077 year: 2006 end-page: 1089 ident: bb0465 article-title: The Sm-protein methyltransferase, dart5, is essential for germ-cell specification and maintenance publication-title: Curr. Biol. – volume: 592 start-page: 1400 year: 2018 end-page: 1411 ident: bb0195 article-title: Gemin5 plays a role in unassembled-U1 sn RNA disposal in SMN -deficient cells publication-title: FEBS Lett. – volume: 317 start-page: 2835 year: 2011 end-page: 2844 ident: bb0040 article-title: U bodies respond to nutrient stress in Drosophila publication-title: Exp. Cell Res. – volume: 16 year: 2022 ident: bb0480 article-title: Identification of a cytokine profile in serum and cerebrospinal fluid of pediatric and adult spinal muscular atrophy patients and its modulation upon nusinersen treatment publication-title: Front. Cell. Neurosci. – volume: 42 year: 2023 ident: bb0225 article-title: Nucleic acid-protein condensates in innate immune signaling publication-title: EMBO J. – volume: 48 start-page: 10456 year: 2020 end-page: 10469 ident: bb0130 article-title: Affinity proteomic dissection of the human nuclear cap-binding complex interactome publication-title: Nucleic Acids Res. – volume: 26 year: 2023 ident: bb0375 article-title: Mechanism of assembly of snRNP cores assisted by ICln and the SMN complex in fission yeast publication-title: IScience – volume: 9 start-page: 2869 year: 2000 end-page: 2877 ident: bb0355 article-title: The exon 2b region of the spinal muscular atrophy protein, SMN, is involved in self-association and SIP1 binding publication-title: Hum. Mol. Genet. – volume: 41 start-page: 140 year: 2021 end-page: 153 ident: bb0425 article-title: Dicer-independent snRNA/snoRNA-derived nuclear RNA 3 regulates tumor-associated macrophage function by epigenetically repressing inducible nitric oxide synthase transcription publication-title: Cancer Commun. (Lond.) – volume: 147 start-page: 690 year: 2011 end-page: 703 ident: bb0450 article-title: A protein complex network of publication-title: Cell – volume: 52 start-page: 131 year: 2018 end-page: 157 ident: bb0385 article-title: PiRNA-guided genome defense: from biogenesis to silencing publication-title: Annu. Rev. Genet. – volume: 15 start-page: 108 year: 2014 end-page: 121 ident: bb0075 article-title: A day in the life of the spliceosome publication-title: Nat. Rev. Mol. Cell Biol. – volume: 27 start-page: 4061 year: 2018 end-page: 4076 ident: bb0475 article-title: A severe mouse model of spinal muscular atrophy develops early systemic inflammation publication-title: Hum. Mol. Genet. – volume: 440 start-page: 41 year: 2006 end-page: 45 ident: bb0230 article-title: Introns and the origin of nucleus-cytosol compartmentalization publication-title: Nature – volume: 14 start-page: 226 year: 2004 end-page: 232 ident: bb0215 article-title: Why do cells need an assembly machine for RNA–protein complexes? publication-title: Trends Cell Biol. – volume: 21 year: 2020 ident: bb0125 article-title: Emerging roles of Gemin5: from snRNPs assembly to translation control publication-title: Int. J. Mol. Sci. – volume: 39 start-page: 24 year: 2017 end-page: 33 ident: bb0435 article-title: Emergent properties of bacterial chemotaxis pathway publication-title: Curr. Opin. Microbiol. – volume: 30 start-page: 12005 year: 2010 end-page: 12019 ident: bb0470 article-title: Reduced survival of motor neuron (SMN) protein in motor neuronal progenitors functions cell autonomously to cause spinal muscular atrophy in model mice expressing the human centromeric (SMN2) gene publication-title: J. Neurosci. – volume: 290 start-page: 20904 year: 2015 end-page: 20918 ident: bb0035 article-title: Intracellular bacterial pathogens trigger the formation of U small nuclear RNA bodies (U bodies) through metabolic stress induction publication-title: J. Biol. Chem. – volume: 48 start-page: 6184 year: 2020 end-page: 6197 ident: bb0180 article-title: DIS3L2 and LSm proteins are involved in the surveillance of Sm ring-deficient snRNAs publication-title: Nucleic Acids Res. – volume: 22 year: 2021 ident: bb0415 article-title: Dynamic subcellular compartmentalization ensures fidelity of piRNA biogenesis in silkworms publication-title: EMBO Rep. – volume: 34 start-page: 989 year: 2020 end-page: 1001 ident: bb0185 article-title: Competition between maturation and degradation drives human snRNA 3′ end quality control publication-title: Genes Dev. – volume: 10 start-page: 597 year: 2009 end-page: 609 ident: bb0360 article-title: Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick? publication-title: Nat. Rev. Neurosci. – volume: 67 start-page: 41 year: 2021 end-page: 50 ident: bb0010 article-title: How do intrinsically disordered protein regions encode a driving force for liquid–liquid phase separation? publication-title: Curr. Opin. Struct. Biol. – volume: 20 start-page: 1929 year: 2012 end-page: 1939 ident: bb0330 article-title: The survival motor neuron protein forms soluble glycine zipper oligomers publication-title: Structure – volume: 121 year: 2024 ident: bb0105 article-title: Sm complex assembly and 5′ cap trimethylation promote selective processing of snRNAs by the 3′ exonuclease TOE1 publication-title: Proc. Natl. Acad. Sci. USA – volume: 344 start-page: 169 year: 2011 end-page: 181 ident: bb0420 article-title: Nuage morphogenesis becomes more complex: two translocation pathways and two forms of nuage coexist in Drosophila germline syncytia publication-title: Cell Tissue Res. – volume: 106 start-page: 11901 year: 2009 end-page: 11906 ident: bb0240 article-title: Protein-free small nuclear RNAs catalyze a two-step splicing reaction publication-title: Proc. Natl. Acad. Sci. USA – volume: 14 start-page: 701 year: 2017 end-page: 711 ident: bb0370 article-title: SMN - a chaperone for nuclear RNP social occasions? publication-title: RNA Biol. – volume: 21 start-page: 8289 year: 2001 end-page: 8300 ident: bb0140 article-title: The methylosome, a 20S complex containing JBP1 and pICln, produces dimethylarginine-modified Sm proteins publication-title: Mol. Cell. Biol. – volume: 101 start-page: 187 year: 2000 end-page: 198 ident: bb0110 article-title: PHAX, a mediator of U snRNA nuclear export whose activity is regulated by phosphorylation publication-title: Cell – volume: 19 start-page: 2244 year: 2017 end-page: 2256 ident: bb0525 article-title: Evidence that C9ORF72 dipeptide repeat proteins associate with U2 snRNP to cause mis-splicing in ALS/FTD patients publication-title: Cell Rep. – volume: 4 start-page: 387 year: 2004 end-page: 393 ident: bb0060 article-title: Distributions of exons and introns in the human genome publication-title: Silico Biol. – volume: 49 start-page: 6437 year: 2021 end-page: 6455 ident: bb0210 article-title: An essential role of the autophagy activating kinase ULK1 in snRNP biogenesis publication-title: Nucleic Acids Res. – volume: 49 start-page: 7644 year: 2021 end-page: 7664 ident: bb0340 article-title: Assembly of higher-order SMN oligomers is essential for metazoan viability and requires an exposed structural motif present in the YG zipper dimer publication-title: Nucleic Acids Res. – volume: 27 start-page: 1822 year: 2019 end-page: 1835 ident: bb0405 article-title: Distinct and collaborative functions of Yb and Armitage in transposon-targeting piRNA biogenesis publication-title: Cell Rep. – volume: 12 start-page: 629 year: 2011 end-page: 642 ident: bb0380 article-title: Deciphering arginine methylation: Tudor tells the tale publication-title: Nat. Rev. Mol. Cell Biol. – volume: 42 start-page: 2708 year: 2014 end-page: 2724 ident: bb0135 article-title: Identification of truncated forms of U1 snRNA reveals a novel RNA degradation pathway during snRNP biogenesis publication-title: Nucleic Acids Res. – volume: 28 start-page: 487 year: 2008 end-page: 497 ident: bb0120 article-title: A compartmentalized phosphorylation/dephosphorylation system that regulates U snRNA export from the nucleus publication-title: Mol. Cell. Biol. – volume: 11 year: 2020 ident: bb0235 article-title: Retroelement origins of pre-mRNA splicing publication-title: Wiley Interdiscip. Rev. RNA – year: 2024 ident: bb0395 article-title: TDRD1 phase separation drives intermitochondrial cement assembly to promote piRNA biogenesis and fertility publication-title: Dev. Cell – volume: 184 start-page: 3612 year: 2021 end-page: 3625 ident: bb0365 article-title: DMA-tudor interaction modules control the specificity of in vivo condensates publication-title: Cell – volume: 137 year: 2024 ident: bb0305 article-title: Dissecting the role of SMN multimerization in its dissociation from the Cajal body using harmine as a tool compound publication-title: J. Cell Sci. – volume: 9 start-page: 531 year: 2018 ident: 10.1016/j.bbagen.2025.130764_bb0270 article-title: Nucleotide modifications decrease innate immune response induced by synthetic analogs of snRNAs and snoRNAs publication-title: Genes (Basel) doi: 10.3390/genes9110531 – volume: 12 start-page: 629 year: 2011 ident: 10.1016/j.bbagen.2025.130764_bb0380 article-title: Deciphering arginine methylation: Tudor tells the tale publication-title: Nat. Rev. Mol. Cell Biol. doi: 10.1038/nrm3185 – volume: 10 year: 2019 ident: 10.1016/j.bbagen.2025.130764_bb0255 article-title: RNAs in the spliceosome: insight from cryoEM structures publication-title: Wiley Interdiscip. Rev. RNA doi: 10.1002/wrna.1523 – volume: 20 start-page: 89 year: 2019 ident: 10.1016/j.bbagen.2025.130764_bb0390 article-title: PIWI-interacting RNAs: small RNAs with big functions publication-title: Nat. Rev. Genet. doi: 10.1038/s41576-018-0073-3 – volume: 49 start-page: 692 year: 2013 ident: 10.1016/j.bbagen.2025.130764_bb0150 article-title: Structural basis of assembly chaperone- mediated snRNP formation publication-title: Mol. Cell doi: 10.1016/j.molcel.2012.12.009 – volume: 1868 year: 2021 ident: 10.1016/j.bbagen.2025.130764_bb0015 article-title: Liquid-liquid phase separation as a common organizing principle of intracellular space and biomembranes providing dynamic adaptive responses publication-title: Biochim. Biophys. Acta, Mol. Cell Res. doi: 10.1016/j.bbamcr.2021.119102 – volume: 11 year: 2020 ident: 10.1016/j.bbagen.2025.130764_bb0235 article-title: Retroelement origins of pre-mRNA splicing publication-title: Wiley Interdiscip. Rev. RNA doi: 10.1002/wrna.1589 – volume: 2 start-page: 718 year: 2011 ident: 10.1016/j.bbagen.2025.130764_bb0070 article-title: Biogenesis of spliceosomal small nuclear ribonucleoproteins: biogenesis of spliceosomal snRNPs publication-title: Wiley Interdiscip. Rev. RNA doi: 10.1002/wrna.87 – volume: 50 start-page: D439 year: 2022 ident: 10.1016/j.bbagen.2025.130764_bb0315 article-title: AlphaFold protein structure database: massively expanding the structural coverage of protein-sequence space with high-accuracy models publication-title: Nucleic Acids Res. doi: 10.1093/nar/gkab1061 – volume: 244 year: 2024 ident: 10.1016/j.bbagen.2025.130764_bb0490 article-title: Cytokines in cerebrospinal fluid as a prognostic predictor after treatment of nusinersen in SMA patients publication-title: Clin. Neurol. Neurosurg. doi: 10.1016/j.clineuro.2024.108462 – volume: 26 start-page: 801 year: 2017 ident: 10.1016/j.bbagen.2025.130764_bb0495 article-title: Immune dysregulation may contribute to disease pathogenesis in spinal muscular atrophy mice publication-title: Hum. Mol. Genet. – volume: 332 start-page: 142 year: 2009 ident: 10.1016/j.bbagen.2025.130764_bb0290 article-title: The spinal muscular atrophy protein SMN affects Drosophila germline nuclear organization through the U body–P body pathway publication-title: Dev. Biol. doi: 10.1016/j.ydbio.2009.05.553 – volume: 48 start-page: 6184 year: 2020 ident: 10.1016/j.bbagen.2025.130764_bb0180 article-title: DIS3L2 and LSm proteins are involved in the surveillance of Sm ring-deficient snRNAs publication-title: Nucleic Acids Res. doi: 10.1093/nar/gkaa301 – volume: 32 start-page: 1077 year: 2010 ident: 10.1016/j.bbagen.2025.130764_bb0065 article-title: SMN and Gemins: “we are family” … or are we?: insights into the partnership between Gemins and the spinal muscular atrophy disease protein SMN: insights into the partnership between Gemins and the spinal muscular atrophy disease protein SMN publication-title: Bioessays doi: 10.1002/bies.201000088 – volume: 3 year: 2011 ident: 10.1016/j.bbagen.2025.130764_bb0045 article-title: Spliceosome structure and function publication-title: Cold Spring Harb. Perspect. Biol. doi: 10.1101/cshperspect.a003707 – volume: 42 start-page: 2708 year: 2014 ident: 10.1016/j.bbagen.2025.130764_bb0135 article-title: Identification of truncated forms of U1 snRNA reveals a novel RNA degradation pathway during snRNP biogenesis publication-title: Nucleic Acids Res. doi: 10.1093/nar/gkt1271 – year: 2024 ident: 10.1016/j.bbagen.2025.130764_bb0395 article-title: TDRD1 phase separation drives intermitochondrial cement assembly to promote piRNA biogenesis and fertility publication-title: Dev. Cell doi: 10.1016/j.devcel.2024.06.017 – volume: 16 start-page: 3103 year: 2016 ident: 10.1016/j.bbagen.2025.130764_bb0145 article-title: The ribosome cooperates with the assembly chaperone pICln to initiate formation of snRNPs publication-title: Cell Rep. doi: 10.1016/j.celrep.2016.08.047 – volume: 42 year: 2023 ident: 10.1016/j.bbagen.2025.130764_bb0225 article-title: Nucleic acid-protein condensates in innate immune signaling publication-title: EMBO J. doi: 10.15252/embj.2022111870 – volume: 43 start-page: 3208 year: 2015 ident: 10.1016/j.bbagen.2025.130764_bb0510 article-title: U1 snRNP is mislocalized in ALS patient fibroblasts bearing NLS mutations in FUS and is required for motor neuron outgrowth in zebrafish publication-title: Nucleic Acids Res. doi: 10.1093/nar/gkv157 – volume: 22 start-page: 1215 year: 2016 ident: 10.1016/j.bbagen.2025.130764_bb0440 article-title: Transcriptomic comparison of Drosophila snRNP biogenesis mutants reveals mutant-specific changes in pre-mRNA processing: implications for spinal muscular atrophy publication-title: RNA doi: 10.1261/rna.057208.116 – volume: 137 year: 2024 ident: 10.1016/j.bbagen.2025.130764_bb0305 article-title: Dissecting the role of SMN multimerization in its dissociation from the Cajal body using harmine as a tool compound publication-title: J. Cell Sci. doi: 10.1242/jcs.261834 – volume: 3 start-page: 28 year: 2023 ident: 10.1016/j.bbagen.2025.130764_bb0485 article-title: Nusinersen mitigates neuroinflammation in severe spinal muscular atrophy patients publication-title: Commun. Med. (Lond.) doi: 10.1038/s43856-023-00256-2 – volume: 106 start-page: 11901 year: 2009 ident: 10.1016/j.bbagen.2025.130764_bb0240 article-title: Protein-free small nuclear RNAs catalyze a two-step splicing reaction publication-title: Proc. Natl. Acad. Sci. USA doi: 10.1073/pnas.0902020106 – volume: 8 start-page: 775 year: 1999 ident: 10.1016/j.bbagen.2025.130764_bb0350 article-title: The RNA-binding properties of SMN: deletion analysis of the zebrafish orthologue defines domains conserved in evolution publication-title: Hum. Mol. Genet. doi: 10.1093/hmg/8.5.775 – volume: 22 start-page: 94 year: 2024 ident: 10.1016/j.bbagen.2025.130764_bb0445 article-title: Dysregulation of innate immune signaling in animal models of spinal muscular atrophy publication-title: BMC Biol. doi: 10.1186/s12915-024-01888-z – volume: 399 start-page: 1265 year: 2018 ident: 10.1016/j.bbagen.2025.130764_bb0100 article-title: Modifications in small nuclear RNAs and their roles in spliceosome assembly and function publication-title: Biol. Chem. doi: 10.1515/hsz-2018-0205 – volume: 67 start-page: 41 year: 2021 ident: 10.1016/j.bbagen.2025.130764_bb0010 article-title: How do intrinsically disordered protein regions encode a driving force for liquid–liquid phase separation? publication-title: Curr. Opin. Struct. Biol. doi: 10.1016/j.sbi.2020.09.004 – volume: 146 start-page: 384 year: 2011 ident: 10.1016/j.bbagen.2025.130764_bb0160 article-title: Structure of a key intermediate of the SMN complex reveals Gemin2’s crucial function in snRNP assembly publication-title: Cell doi: 10.1016/j.cell.2011.06.043 – volume: 7 start-page: 26496 year: 2016 ident: 10.1016/j.bbagen.2025.130764_bb0260 article-title: Cancer therapies activate RIG-I-like receptor pathway through endogenous non-coding RNAs publication-title: Oncotarget doi: 10.18632/oncotarget.8420 – volume: 12 year: 2021 ident: 10.1016/j.bbagen.2025.130764_bb0095 article-title: Spliceosomal snRNA Epitranscriptomics publication-title: Front. Genet. doi: 10.3389/fgene.2021.652129 – volume: 5 start-page: 528 year: 2015 ident: 10.1016/j.bbagen.2025.130764_bb0455 article-title: Gemin5: a multitasking RNA-binding protein involved in translation control publication-title: Biomolecules doi: 10.3390/biom5020528 – volume: 19 start-page: 2244 year: 2017 ident: 10.1016/j.bbagen.2025.130764_bb0525 article-title: Evidence that C9ORF72 dipeptide repeat proteins associate with U2 snRNP to cause mis-splicing in ALS/FTD patients publication-title: Cell Rep. doi: 10.1016/j.celrep.2017.05.056 – volume: 121 year: 2024 ident: 10.1016/j.bbagen.2025.130764_bb0105 article-title: Sm complex assembly and 5′ cap trimethylation promote selective processing of snRNAs by the 3′ exonuclease TOE1 publication-title: Proc. Natl. Acad. Sci. USA – volume: 57 start-page: 936 year: 2015 ident: 10.1016/j.bbagen.2025.130764_bb0400 article-title: Phase transition of a disordered nuage protein generates environmentally responsive membraneless organelles publication-title: Mol. Cell doi: 10.1016/j.molcel.2015.01.013 – volume: 7 start-page: 1269 year: 1998 ident: 10.1016/j.bbagen.2025.130764_bb0345 article-title: The domain encoded by exon 2 of the survival motor neuron protein mediates nucleic acid binding publication-title: Hum. Mol. Genet. doi: 10.1093/hmg/7.8.1269 – volume: 53 start-page: 26 year: 2020 ident: 10.1016/j.bbagen.2025.130764_bb0220 article-title: Distinct and orchestrated functions of RNA sensors in innate immunity publication-title: Immunity doi: 10.1016/j.immuni.2020.03.017 – volume: 127 start-page: 3909 year: 2014 ident: 10.1016/j.bbagen.2025.130764_bb0275 article-title: The splicing factor U1-70K interacts with the SMN complex and is required for nuclear gem integrity publication-title: J. Cell Sci. – volume: 14 start-page: 701 year: 2017 ident: 10.1016/j.bbagen.2025.130764_bb0370 article-title: SMN - a chaperone for nuclear RNP social occasions? publication-title: RNA Biol. doi: 10.1080/15476286.2016.1236168 – volume: 39 start-page: 24 year: 2017 ident: 10.1016/j.bbagen.2025.130764_bb0435 article-title: Emergent properties of bacterial chemotaxis pathway publication-title: Curr. Opin. Microbiol. doi: 10.1016/j.mib.2017.07.004 – volume: 31 year: 2022 ident: 10.1016/j.bbagen.2025.130764_bb0320 article-title: Intrinsic protein disorder and conditional folding in AlphaFoldDB publication-title: Protein Sci. doi: 10.1002/pro.4466 – volume: 27 start-page: 1822 year: 2019 ident: 10.1016/j.bbagen.2025.130764_bb0405 article-title: Distinct and collaborative functions of Yb and Armitage in transposon-targeting piRNA biogenesis publication-title: Cell Rep. doi: 10.1016/j.celrep.2019.04.029 – volume: 20 start-page: 1929 year: 2012 ident: 10.1016/j.bbagen.2025.130764_bb0330 article-title: The survival motor neuron protein forms soluble glycine zipper oligomers publication-title: Structure doi: 10.1016/j.str.2012.08.024 – volume: 101 start-page: 187 year: 2000 ident: 10.1016/j.bbagen.2025.130764_bb0110 article-title: PHAX, a mediator of U snRNA nuclear export whose activity is regulated by phosphorylation publication-title: Cell doi: 10.1016/S0092-8674(00)80829-6 – volume: 10 start-page: 597 year: 2009 ident: 10.1016/j.bbagen.2025.130764_bb0360 article-title: Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick? publication-title: Nat. Rev. Neurosci. doi: 10.1038/nrn2670 – volume: 111 start-page: E3277 year: 2014 ident: 10.1016/j.bbagen.2025.130764_bb0175 article-title: Quality control of assembly-defective U1 snRNAs by decapping and 5′-to-3′ exonucleolytic digestion publication-title: Proc. Natl. Acad. Sci. USA doi: 10.1073/pnas.1412614111 – volume: 440 start-page: 41 year: 2006 ident: 10.1016/j.bbagen.2025.130764_bb0230 article-title: Introns and the origin of nucleus-cytosol compartmentalization publication-title: Nature doi: 10.1038/nature04531 – volume: 49 start-page: 7644 year: 2021 ident: 10.1016/j.bbagen.2025.130764_bb0340 article-title: Assembly of higher-order SMN oligomers is essential for metazoan viability and requires an exposed structural motif present in the YG zipper dimer publication-title: Nucleic Acids Res. doi: 10.1093/nar/gkab508 – volume: 30 start-page: 12005 year: 2010 ident: 10.1016/j.bbagen.2025.130764_bb0470 article-title: Reduced survival of motor neuron (SMN) protein in motor neuronal progenitors functions cell autonomously to cause spinal muscular atrophy in model mice expressing the human centromeric (SMN2) gene publication-title: J. Neurosci. doi: 10.1523/JNEUROSCI.2208-10.2010 – year: 2022 ident: 10.1016/j.bbagen.2025.130764_bb0025 article-title: A guide to membraneless organelles and their various roles in gene regulation publication-title: Nat. Rev. Mol. Cell Biol. – volume: 104 start-page: 11655 year: 2007 ident: 10.1016/j.bbagen.2025.130764_bb0030 article-title: U bodies are cytoplasmic structures that contain uridine-rich small nuclear ribonucleoproteins and associate with P bodies publication-title: Proc. Natl. Acad. Sci. USA doi: 10.1073/pnas.0704977104 – volume: 41 start-page: 140 year: 2021 ident: 10.1016/j.bbagen.2025.130764_bb0425 article-title: Dicer-independent snRNA/snoRNA-derived nuclear RNA 3 regulates tumor-associated macrophage function by epigenetically repressing inducible nitric oxide synthase transcription publication-title: Cancer Commun. (Lond.) doi: 10.1002/cac2.12131 – volume: 34 start-page: 989 year: 2020 ident: 10.1016/j.bbagen.2025.130764_bb0185 article-title: Competition between maturation and degradation drives human snRNA 3′ end quality control publication-title: Genes Dev. doi: 10.1101/gad.336891.120 – volume: 52 start-page: 131 year: 2018 ident: 10.1016/j.bbagen.2025.130764_bb0385 article-title: PiRNA-guided genome defense: from biogenesis to silencing publication-title: Annu. Rev. Genet. doi: 10.1146/annurev-genet-120417-031441 – volume: 21 start-page: 8289 year: 2001 ident: 10.1016/j.bbagen.2025.130764_bb0140 article-title: The methylosome, a 20S complex containing JBP1 and pICln, produces dimethylarginine-modified Sm proteins publication-title: Mol. Cell. Biol. doi: 10.1128/MCB.21.24.8289-8300.2001 – volume: 290 start-page: 20185 year: 2015 ident: 10.1016/j.bbagen.2025.130764_bb0335 article-title: Oligomeric properties of survival motor neuron·Gemin2 complexes publication-title: J. Biol.Chem. doi: 10.1074/jbc.M115.667279 – volume: 14 start-page: 693 year: 2017 ident: 10.1016/j.bbagen.2025.130764_bb0090 article-title: RNA modification in Cajal bodies publication-title: RNA Biol. doi: 10.1080/15476286.2016.1249091 – volume: 174 start-page: 791 year: 2018 ident: 10.1016/j.bbagen.2025.130764_bb0005 article-title: Emerging roles for intermolecular RNA-RNA interactions in RNP assemblies publication-title: Cell doi: 10.1016/j.cell.2018.07.023 – volume: 6 start-page: 6171 year: 2015 ident: 10.1016/j.bbagen.2025.130764_bb0515 article-title: ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP publication-title: Nat. Commun. doi: 10.1038/ncomms7171 – volume: 89 start-page: 359 year: 2020 ident: 10.1016/j.bbagen.2025.130764_bb0055 article-title: RNA splicing by the spliceosome publication-title: Annu. Rev. Biochem. doi: 10.1146/annurev-biochem-091719-064225 – volume: 27 start-page: 3199 year: 2019 ident: 10.1016/j.bbagen.2025.130764_bb0085 article-title: Nuclear pre-snRNA export is an essential quality assurance mechanism for functional spliceosomes publication-title: Cell Rep. doi: 10.1016/j.celrep.2019.05.031 – volume: 27 start-page: 4061 year: 2018 ident: 10.1016/j.bbagen.2025.130764_bb0475 article-title: A severe mouse model of spinal muscular atrophy develops early systemic inflammation publication-title: Hum. Mol. Genet. – volume: 28 start-page: 487 year: 2008 ident: 10.1016/j.bbagen.2025.130764_bb0120 article-title: A compartmentalized phosphorylation/dephosphorylation system that regulates U snRNA export from the nucleus publication-title: Mol. Cell. Biol. doi: 10.1128/MCB.01189-07 – volume: 25 start-page: 2255 year: 2008 ident: 10.1016/j.bbagen.2025.130764_bb0165 article-title: Evolutionary diversification of the Sm family of RNA-associated proteins publication-title: Mol. Biol. Evol. doi: 10.1093/molbev/msn175 – volume: 4 start-page: 387 year: 2004 ident: 10.1016/j.bbagen.2025.130764_bb0060 article-title: Distributions of exons and introns in the human genome publication-title: Silico Biol. doi: 10.3233/ISB-00142 – volume: 94 start-page: 245 year: 2016 ident: 10.1016/j.bbagen.2025.130764_bb0205 article-title: Disruption of snRNP biogenesis factors Tgs1 and pICln induces phenotypes that mirror aspects of SMN-Gemins complex perturbation in Drosophila, providing new insights into spinal muscular atrophy publication-title: Neurobiol. Dis. doi: 10.1016/j.nbd.2016.06.015 – volume: 22 year: 2021 ident: 10.1016/j.bbagen.2025.130764_bb0415 article-title: Dynamic subcellular compartmentalization ensures fidelity of piRNA biogenesis in silkworms publication-title: EMBO Rep. doi: 10.15252/embr.202051342 – volume: 151 start-page: 497 year: 2012 ident: 10.1016/j.bbagen.2025.130764_bb0245 article-title: Visualizing group II intron catalysis through the stages of splicing publication-title: Cell doi: 10.1016/j.cell.2012.09.033 – volume: 16 year: 2020 ident: 10.1016/j.bbagen.2025.130764_bb0410 article-title: Adaptive evolution among cytoplasmic piRNA proteins leads to decreased genomic auto-immunity publication-title: PLoS Genet. doi: 10.1371/journal.pgen.1008861 – volume: 10 year: 2015 ident: 10.1016/j.bbagen.2025.130764_bb0200 article-title: Genetic interactions between the members of the SMN-Gemins complex in Drosophila publication-title: PLoS One doi: 10.1371/journal.pone.0130974 – volume: 9 start-page: 891 year: 2002 ident: 10.1016/j.bbagen.2025.130764_bb0080 article-title: Hypermethylation of the cap structure of both yeast snRNAs and snoRNAs requires a conserved methyltransferase that is localized to the nucleolus publication-title: Mol. Cell doi: 10.1016/S1097-2765(02)00484-7 – volume: 21 year: 2020 ident: 10.1016/j.bbagen.2025.130764_bb0125 article-title: Emerging roles of Gemin5: from snRNPs assembly to translation control publication-title: Int. J. Mol. Sci. doi: 10.3390/ijms21113868 – volume: 120 year: 2023 ident: 10.1016/j.bbagen.2025.130764_bb0325 article-title: Systematic identification of conditionally folded intrinsically disordered regions by AlphaFold2 publication-title: Proc. Natl. Acad. Sci. USA doi: 10.1073/pnas.2304302120 – volume: 335 start-page: 1643 year: 2012 ident: 10.1016/j.bbagen.2025.130764_bb0115 article-title: hnRNP C tetramer measures RNA length to classify RNA polymerase II transcripts for export publication-title: Science doi: 10.1126/science.1218469 – volume: 9 start-page: 2869 year: 2000 ident: 10.1016/j.bbagen.2025.130764_bb0355 article-title: The exon 2b region of the spinal muscular atrophy protein, SMN, is involved in self-association and SIP1 binding publication-title: Hum. Mol. Genet. doi: 10.1093/hmg/9.19.2869 – volume: 11 start-page: 6341 year: 2020 ident: 10.1016/j.bbagen.2025.130764_bb0520 article-title: Aberrant interaction of FUS with the U1 snRNA provides a molecular mechanism of FUS induced amyotrophic lateral sclerosis publication-title: Nat. Commun. doi: 10.1038/s41467-020-20191-3 – volume: 14 start-page: 226 year: 2004 ident: 10.1016/j.bbagen.2025.130764_bb0215 article-title: Why do cells need an assembly machine for RNA–protein complexes? publication-title: Trends Cell Biol. doi: 10.1016/j.tcb.2004.03.010 – volume: 35 start-page: 1233 year: 2011 ident: 10.1016/j.bbagen.2025.130764_bb0280 article-title: Gem formation upon constitutive Gemin3 overexpression in Drosophila publication-title: Cell Biol. Int. doi: 10.1042/CBI20110147 – volume: 290 start-page: 20904 year: 2015 ident: 10.1016/j.bbagen.2025.130764_bb0035 article-title: Intracellular bacterial pathogens trigger the formation of U small nuclear RNA bodies (U bodies) through metabolic stress induction publication-title: J. Biol. Chem. doi: 10.1074/jbc.M115.659466 – volume: 14 start-page: 878 year: 2008 ident: 10.1016/j.bbagen.2025.130764_bb0285 article-title: Sm protein methylation is dispensable for snRNP assembly in Drosophila melanogaster publication-title: RNA doi: 10.1261/rna.940708 – volume: 95 start-page: 615 year: 1998 ident: 10.1016/j.bbagen.2025.130764_bb0300 article-title: A novel function for SMN, the spinal muscular atrophy disease gene product, in pre-mRNA splicing publication-title: Cell doi: 10.1016/S0092-8674(00)81632-3 – volume: 316 start-page: 2354 year: 2010 ident: 10.1016/j.bbagen.2025.130764_bb0295 article-title: Drosophila SMN complex proteins Gemin2, Gemin3, and Gemin5 are components of U bodies publication-title: Exp. Cell Res. doi: 10.1016/j.yexcr.2010.05.001 – volume: 16 year: 2022 ident: 10.1016/j.bbagen.2025.130764_bb0480 article-title: Identification of a cytokine profile in serum and cerebrospinal fluid of pediatric and adult spinal muscular atrophy patients and its modulation upon nusinersen treatment publication-title: Front. Cell. Neurosci. doi: 10.3389/fncel.2022.982760 – volume: 29 start-page: 3087 year: 2019 ident: 10.1016/j.bbagen.2025.130764_bb0505 article-title: The classical complement pathway mediates microglia-dependent remodeling of spinal motor circuits during development and in SMA publication-title: Cell Rep. doi: 10.1016/j.celrep.2019.11.013 – volume: 344 start-page: 169 year: 2011 ident: 10.1016/j.bbagen.2025.130764_bb0420 article-title: Nuage morphogenesis becomes more complex: two translocation pathways and two forms of nuage coexist in Drosophila germline syncytia publication-title: Cell Tissue Res. doi: 10.1007/s00441-011-1145-2 – volume: 26 year: 2023 ident: 10.1016/j.bbagen.2025.130764_bb0375 article-title: Mechanism of assembly of snRNP cores assisted by ICln and the SMN complex in fission yeast publication-title: IScience doi: 10.1016/j.isci.2023.107604 – volume: 147 start-page: 690 year: 2011 ident: 10.1016/j.bbagen.2025.130764_bb0450 article-title: A protein complex network of Drosophila melanogaster publication-title: Cell doi: 10.1016/j.cell.2011.08.047 – volume: 12 year: 2021 ident: 10.1016/j.bbagen.2025.130764_bb0460 article-title: Uncovering targets of the leader protease: linking RNA-mediated pathways and antiviral defense publication-title: Wiley Interdiscip. Rev. RNA doi: 10.1002/wrna.1645 – volume: 15 start-page: 108 year: 2014 ident: 10.1016/j.bbagen.2025.130764_bb0075 article-title: A day in the life of the spliceosome publication-title: Nat. Rev. Mol. Cell Biol. doi: 10.1038/nrm3742 – volume: 25 start-page: 4717 year: 2016 ident: 10.1016/j.bbagen.2025.130764_bb0310 article-title: A critical examination of the recently reported crystal structures of the human SMN protein publication-title: Hum. Mol. Genet. – volume: 34 start-page: 1925 year: 2015 ident: 10.1016/j.bbagen.2025.130764_bb0155 article-title: Reconstitution of the human U snRNP assembly machinery reveals stepwise Sm protein organization publication-title: EMBO J. doi: 10.15252/embj.201490350 – volume: 1 year: 2021 ident: 10.1016/j.bbagen.2025.130764_bb0430 article-title: Global analysis of protein arginine methylation publication-title: Cell Rep. Methods – volume: 26 start-page: 932 year: 2017 ident: 10.1016/j.bbagen.2025.130764_bb0500 article-title: SMN deficiency negatively impacts red pulp macrophages and spleen development in mouse models of spinal muscular atrophy publication-title: Hum. Mol. Genet. – volume: 216 start-page: 2391 year: 2017 ident: 10.1016/j.bbagen.2025.130764_bb0190 article-title: Impaired spliceosomal UsnRNP assembly leads to Sm mRNA down-regulation and Sm protein degradation publication-title: J. Cell Biol. doi: 10.1083/jcb.201611108 – volume: 43 year: 2024 ident: 10.1016/j.bbagen.2025.130764_bb0265 article-title: Housekeeping U1 snRNA facilitates antiviral innate immunity by promoting TRIM25-mediated RIG-I activation publication-title: Cell Rep. – volume: 20 start-page: 574 year: 2014 ident: 10.1016/j.bbagen.2025.130764_bb0530 article-title: Gem depletion: amyotrophic lateral sclerosis and spinal muscular atrophy crossover publication-title: CNS Neurosci. Ther. doi: 10.1111/cns.12242 – volume: 89 start-page: 333 year: 2020 ident: 10.1016/j.bbagen.2025.130764_bb0050 article-title: How is precursor messenger RNA spliced by the spliceosome? publication-title: Annu. Rev. Biochem. doi: 10.1146/annurev-biochem-013118-111024 – volume: 21 start-page: 464 year: 2014 ident: 10.1016/j.bbagen.2025.130764_bb0250 article-title: Evidence for a group II intron-like catalytic triplex in the spliceosome publication-title: Nat. Struct. Mol. Biol. doi: 10.1038/nsmb.2815 – volume: 16 start-page: 1077 year: 2006 ident: 10.1016/j.bbagen.2025.130764_bb0465 article-title: The Sm-protein methyltransferase, dart5, is essential for germ-cell specification and maintenance publication-title: Curr. Biol. doi: 10.1016/j.cub.2006.04.037 – volume: 48 start-page: 10456 year: 2020 ident: 10.1016/j.bbagen.2025.130764_bb0130 article-title: Affinity proteomic dissection of the human nuclear cap-binding complex interactome publication-title: Nucleic Acids Res. doi: 10.1093/nar/gkaa743 – volume: 184 start-page: 3612 year: 2021 ident: 10.1016/j.bbagen.2025.130764_bb0365 article-title: DMA-tudor interaction modules control the specificity of in vivo condensates publication-title: Cell doi: 10.1016/j.cell.2021.05.008 – volume: 614 start-page: 358 year: 2023 ident: 10.1016/j.bbagen.2025.130764_bb0170 article-title: mRNA ageing shapes the Cap2 methylome in mammalian mRNA publication-title: Nature doi: 10.1038/s41586-022-05668-z – volume: 317 start-page: 2835 year: 2011 ident: 10.1016/j.bbagen.2025.130764_bb0040 article-title: U bodies respond to nutrient stress in Drosophila publication-title: Exp. Cell Res. doi: 10.1016/j.yexcr.2011.09.001 – volume: 49 start-page: 6437 year: 2021 ident: 10.1016/j.bbagen.2025.130764_bb0210 article-title: An essential role of the autophagy activating kinase ULK1 in snRNP biogenesis publication-title: Nucleic Acids Res. doi: 10.1093/nar/gkab452 – volume: 592 start-page: 1400 year: 2018 ident: 10.1016/j.bbagen.2025.130764_bb0195 article-title: Gemin5 plays a role in unassembled-U1 sn RNA disposal in SMN -deficient cells publication-title: FEBS Lett. doi: 10.1002/1873-3468.13031 – volume: 28 start-page: 465 year: 2021 ident: 10.1016/j.bbagen.2025.130764_bb0020 article-title: It’s not just a phase: function and characteristics of RNA-binding proteins in phase separation publication-title: Nat. Struct. Mol. Biol. doi: 10.1038/s41594-021-00601-w |
| SSID | ssj0000595 |
| Score | 2.4542232 |
| SecondaryResourceType | review_article |
| Snippet | Biomolecular condensates like U-bodies are specialized cellular structures formed through multivalent interactions among intrinsically disordered regions.... |
| SourceID | proquest pubmed crossref elsevier |
| SourceType | Aggregation Database Index Database Publisher |
| StartPage | 130764 |
| SubjectTerms | Animals biogenesis Biomolecular Condensates - metabolism Cajal body Coilin cytoplasm domain Humans Immunity, Innate inflammation innate immunity Intrinsically disordered region mammals metabolism muscular atrophy Muscular Atrophy, Spinal - genetics Muscular Atrophy, Spinal - immunology Muscular Atrophy, Spinal - metabolism ribonucleoproteins Ribonucleoproteins, Small Nuclear - genetics Ribonucleoproteins, Small Nuclear - immunology Ribonucleoproteins, Small Nuclear - metabolism RNA RNA Processing, Post-Transcriptional SMN SMN Complex Proteins - genetics SMN Complex Proteins - metabolism Symmetrical arginine dimethylation therapeutics Tudor domain U-body |
| Title | Balancing RNA processing and innate immune response: Possible roles for SMN condensates in snRNP biogenesis |
| URI | https://dx.doi.org/10.1016/j.bbagen.2025.130764 https://www.ncbi.nlm.nih.gov/pubmed/39826814 https://www.proquest.com/docview/3157538629 https://www.proquest.com/docview/3165863959 |
| Volume | 1869 |
| hasFullText | 1 |
| inHoldings | 1 |
| isFullTextHit | |
| isPrint | |
| link | http://utb.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwnV1LS8QwEA6iiF7E93uJ4DUubZJ2621dXFbFIj5gbyFJU6lKd3HXgxd_uzNNq3hYBY9JEwgzycw3nRchx1p28ojzkOVSZEw44VgS6oBpLjIecSdM9cPtOo0GD-JyKIdzpNfkwmBYZS37vUyvpHU9066p2R4XRfsOnXoAJ9Avh0ihymAXMd7yk4_vMA-AD9J7EgTD1U36XBXjZQw8WqyCGkpsixxHYpZ6mgU_KzXUXyUrNX6kXX_ENTLnynWy6DtKvq-TpV7TwG2DPJ9h2KIF3URv0y4d-5QAHOoyo0VZAsykBeaHOPrqQ2XdKb0Z4St5gSks9UQB09K765SC2QwSaoLIFLbSSXmb3lBTjB5RVhaTTfLQP7_vDVjdW4FZLsSUxZlwcWKiMNcyyWOrsa4-UMt0nNXWhgkoeq3zSJpIgImSdSIeWzCjRehElgAXt8h8OSrdDqHGBZmBD3kOpqKMrQGbjCcWJEUW6NDaXcIakqqxL6GhmtiyJ-VZoJAFyrNgl8QN3dWPq6BAyv-x86hhkwJao-tDl270NlE8AFjKwXpLflsDaAwAm4Q1257HX-flCZhhnUDs_fts-2QZRz587YDMT1_f3CHgmalpVRe2RRa6F1eD9BPOhPMD |
| linkProvider | Elsevier |
| linkToHtml | http://utb.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwnV1LT9wwEB6hRRVcEFDej7pSr9YqsZ1suC2roqWwEeIhcbNsx6lSUHbFLgf-PTNxQtUDReIYPyRrxh5_XzwPgB9GDcpEiJiXShZceul5FpuIGyELkQgvbfPDbZIn4zv5617dL8Goi4Uht8rW9geb3ljrtqXfSrM_q6r-DT3qIZygdzlCCkiBlik7lerB8vD8Ypz_NciqKb5C4zlN6CLoGjcva_HcUiLUWFFl5DSR791Q7yHQ5iY6W4e1FkKyYVjlBiz5ehO-hKKSL5uwMupquH2Fh1PyXHR4PbHrfMhmISqAPk1dsKquEWmyikJEPHsK3rL-hF1N6aA8YhNle2IIa9nNJGfInNFIzQmc4lQ2r6_zK2ar6W8yl9V8C-7Oft6Oxrwtr8CdkHLB00L6NLNJXBqVlakzlFofpWUH3hnn4gzvemPKRNlEIkspBolIHTJpGXtZZKjIbejV09rvArM-Kix2lCWyRZU6i7RMZA6NRRGZ2Lk94J1I9Sxk0dCde9kfHVSgSQU6qGAP0k7u-p_doNHQfzDze6cmjbKm1w9T--nzXIsIkalAApf9bwwCMsRsCsfsBB2_rVdkyMQGkdz_9Nq-wcr4dnKpL8_ziwNYpZ7gzXYIvcXTsz9CeLOwx-32fQUmuvW0 |
| openUrl | ctx_ver=Z39.88-2004&ctx_enc=info%3Aofi%2Fenc%3AUTF-8&rfr_id=info%3Asid%2Fsummon.serialssolutions.com&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=Balancing+RNA+processing+and+innate+immune+response%3A+Possible+roles+for+SMN+condensates+in+snRNP+biogenesis&rft.jtitle=Biochimica+et+biophysica+acta.+General+subjects&rft.au=Maita%2C+Hiroshi&rft.au=Nakagawa%2C+Shinichi&rft.date=2025-03-01&rft.pub=Elsevier+B.V&rft.issn=0304-4165&rft.volume=1869&rft.issue=3&rft_id=info:doi/10.1016%2Fj.bbagen.2025.130764&rft.externalDocID=S0304416525000091 |
| thumbnail_l | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/lc.gif&issn=0304-4165&client=summon |
| thumbnail_m | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/mc.gif&issn=0304-4165&client=summon |
| thumbnail_s | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/sc.gif&issn=0304-4165&client=summon |