{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T03:39:19Z","timestamp":1790134759510,"version":"4.0.1"},"reference-count":80,"publisher":"Oxford University Press (OUP)","issue":"13","license":[{"start":{"date-parts":[[2021,6,28]],"date-time":"2021-06-28T00:00:00Z","timestamp":1624838400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"NIH","doi-asserted-by":"publisher","award":["R35-GM136435"],"award-info":[{"award-number":["R35-GM136435"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"NIH","doi-asserted-by":"publisher","award":["R01-GM108751"],"award-info":[{"award-number":["R01-GM108751"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"NIH","doi-asserted-by":"publisher","award":["P30-GM124169"],"award-info":[{"award-number":["P30-GM124169"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Johnson Research Foundation"},{"name":"High-End Instrumentation","award":["S10-OD018483"],"award-info":[{"award-number":["S10-OD018483"]}]},{"DOI":"10.13039\/100006231","name":"Brookhaven National Laboratory","doi-asserted-by":"publisher","award":["DE-SC0012704"],"award-info":[{"award-number":["DE-SC0012704"]}],"id":[{"id":"10.13039\/100006231","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,7,21]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Protein oligomerization is one mechanism by which homogenous solutions can separate into distinct liquid phases, enabling assembly of membraneless organelles. Survival Motor Neuron (SMN) is the eponymous component of a large macromolecular complex that chaperones biogenesis of eukaryotic ribonucleoproteins and localizes to distinct membraneless organelles in both the nucleus and cytoplasm. SMN forms the oligomeric core of this complex, and missense mutations within its YG box domain are known to cause Spinal Muscular Atrophy (SMA). The SMN YG box utilizes a unique variant of the glycine zipper motif to form dimers, but the mechanism of higher-order oligomerization remains unknown. Here, we use a combination of molecular genetic, phylogenetic, biophysical, biochemical and computational approaches to show that formation of higher-order SMN oligomers depends on a set of YG box residues that are not involved in dimerization. Mutation of key residues within this new structural motif restricts assembly of SMN to dimers and causes locomotor dysfunction and viability defects in animal models.<\/jats:p>","DOI":"10.1093\/nar\/gkab508","type":"journal-article","created":{"date-parts":[[2021,6,3]],"date-time":"2021-06-03T08:43:20Z","timestamp":1622709800000},"page":"7644-7664","source":"Crossref","is-referenced-by-count":21,"title":["Assembly of higher-order SMN oligomers is essential for metazoan viability and requires an exposed structural motif present in the YG zipper dimer"],"prefix":"10.1093","volume":"49","author":[{"given":"Kushol","family":"Gupta","sequence":"first","affiliation":[{"name":"Department of Biochemistry & Biophysics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19105-6059, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ying","family":"Wen","sequence":"additional","affiliation":[{"name":"Integrative Program for Biological & Genome Sciences, Lineberger Comprehensive Cancer Center, University of North Carolina, Chapel Hill, NC 27599, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nisha S","family":"Ninan","sequence":"additional","affiliation":[{"name":"Department of Biochemistry & Biophysics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19105-6059, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amanda C","family":"Raimer","sequence":"additional","affiliation":[{"name":"Integrative Program for Biological & Genome Sciences, Lineberger Comprehensive Cancer Center, University of North Carolina, Chapel Hill, NC 27599, USA"},{"name":"Curriculum in Genetics and Molecular Biology, Department of Genetics, University of North Carolina, Chapel Hill, NC 27599, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Sharp","sequence":"additional","affiliation":[{"name":"Department of Biochemistry & Biophysics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19105-6059, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashlyn\u00a0M","family":"Spring","sequence":"additional","affiliation":[{"name":"Integrative Program for Biological & Genome Sciences, Lineberger Comprehensive Cancer Center, University of North Carolina, Chapel Hill, NC 27599, USA"},{"name":"Department of Biology, University of North Carolina, Chapel Hill, NC 27599, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathryn L","family":"Sarachan","sequence":"additional","affiliation":[{"name":"Department of Biochemistry & Biophysics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19105-6059, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meghan C","family":"Johnson","sequence":"additional","affiliation":[{"name":"Department of Biology, University of North Carolina, Chapel Hill, NC 27599, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gregory D","family":"Van\u00a0Duyne","sequence":"additional","affiliation":[{"name":"Department of Biochemistry & Biophysics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19105-6059, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6406-0630","authenticated-orcid":false,"given":"A Gregory","family":"Matera","sequence":"additional","affiliation":[{"name":"Integrative Program for Biological & Genome Sciences, Lineberger Comprehensive Cancer Center, University of North Carolina, Chapel Hill, NC 27599, USA"},{"name":"Curriculum in Genetics and Molecular Biology, Department of Genetics, University of North Carolina, Chapel Hill, NC 27599, USA"},{"name":"Department of Biology, University of North Carolina, Chapel Hill, NC 27599, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,6,28]]},"reference":[{"key":"2021071913010577900_B1","doi-asserted-by":"crossref","first-page":"e155","DOI":"10.1371\/journal.pcbi.0020155","article-title":"3D complex: a structural classification of protein complexes","volume":"2","author":"Levy","year":"2006","journal-title":"PLoS Comput. Biol."},{"key":"2021071913010577900_B2","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1080\/07388551.2019.1711014","article-title":"Bioengineering oligomerization and monomerization of enzymes: learning from natural evolution to matching the demands for industrial applications","volume":"40","author":"Liu","year":"2020","journal-title":"Crit. Rev. Biotechnol."},{"key":"2021071913010577900_B3","doi-asserted-by":"crossref","first-page":"5013","DOI":"10.1016\/j.bmc.2005.05.037","article-title":"Protein oligomerization: how and why","volume":"13","author":"Ali","year":"2005","journal-title":"Bioorg. Med. Chem."},{"key":"2021071913010577900_B4","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1038\/nrm3742","article-title":"A day in the life of the spliceosome","volume":"15","author":"Matera","year":"2014","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"2021071913010577900_B5","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1007\/s00412-017-0637-6","article-title":"UsnRNP biogenesis: mechanisms and regulation","volume":"126","author":"Gruss","year":"2017","journal-title":"Chromosoma"},{"key":"2021071913010577900_B6","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1101\/sqb.2006.71.001","article-title":"The SMN complex: an assembly machine for RNPs","volume":"71","author":"Battle","year":"2006","journal-title":"Cold Spring Harb. Symp. Quant. Biol."},{"key":"2021071913010577900_B7","doi-asserted-by":"crossref","first-page":"701","DOI":"10.1080\/15476286.2016.1236168","article-title":"SMN - a chaperone for nuclear RNP social occasions","volume":"14","author":"Raimer","year":"2017","journal-title":"RNA Biol."},{"key":"2021071913010577900_B8","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1016\/j.bbagrm.2016.12.008","article-title":"Diverse role of survival motor neuron protein","volume":"1860","author":"Singh","year":"2017","journal-title":"Biochim. Biophys. Acta"},{"key":"2021071913010577900_B9","doi-asserted-by":"crossref","first-page":"3877","DOI":"10.1007\/s00018-018-2849-1","article-title":"The role of survival motor neuron protein (SMN) in protein homeostasis","volume":"75","author":"Chaytow","year":"2018","journal-title":"Cell. Mol. Life Sci."},{"key":"2021071913010577900_B10","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1007\/978-3-319-89689-2_6","article-title":"RNP assembly defects in spinal muscular atrophy","volume":"20","author":"Price","year":"2018","journal-title":"Adv Neurobiol"},{"key":"2021071913010577900_B11","first-page":"331","article-title":"Membraneless organelles: phasing out of equilibrium","volume":"4","author":"Hondele","year":"2020","journal-title":"Emerg. Top. Life Sci."},{"key":"2021071913010577900_B12","doi-asserted-by":"crossref","first-page":"jcs235093","DOI":"10.1242\/jcs.235093","article-title":"The liquid nucleome - phase transitions in the nucleus at a glance","volume":"132","author":"Strom","year":"2019","journal-title":"J. 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J."},{"key":"2021071913010577900_B28","doi-asserted-by":"crossref","first-page":"943","DOI":"10.1002\/(SICI)1097-0061(199807)14:10<943::AID-YEA292>3.0.CO;2-Y","article-title":"Heterologous modules for efficient and versatile PCR-based gene targeting in Schizosaccharomyces pombe","volume":"14","author":"Bahler","year":"1998","journal-title":"Yeast"},{"key":"2021071913010577900_B29","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/0378-1119(92)90707-V","article-title":"Versatile shuttle vectors and genomic libraries for use with Schizosaccharomyces pombe","volume":"114","author":"Barbet","year":"1992","journal-title":"Gene"},{"key":"2021071913010577900_B30","doi-asserted-by":"crossref","first-page":"2955","DOI":"10.1093\/nar\/21.12.2955","article-title":"Comparison of Schizosaccharomyces pombe expression systems","volume":"21","author":"Forsburg","year":"1993","journal-title":"Nucleic Acids Res."},{"key":"2021071913010577900_B31","doi-asserted-by":"crossref","first-page":"e3209","DOI":"10.1371\/journal.pone.0003209","article-title":"Modeling spinal muscular atrophy in Drosophila","volume":"3","author":"Chang","year":"2008","journal-title":"PLoS One"},{"key":"2021071913010577900_B32","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1083\/jcb.200610053","article-title":"A Drosophila melanogaster model of spinal muscular atrophy reveals a function for SMN in striated muscle","volume":"176","author":"Rajendra","year":"2007","journal-title":"J. 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U.S.A."},{"key":"2021071913010577900_B36","doi-asserted-by":"crossref","first-page":"11167","DOI":"10.1073\/pnas.96.20.11167","article-title":"SMN mutants of spinal muscular atrophy patients are defective in binding to snRNP proteins","volume":"96","author":"Pellizzoni","year":"1999","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"2021071913010577900_B37","doi-asserted-by":"crossref","first-page":"945","DOI":"10.1038\/ncb1101-945","article-title":"A multiprotein complex mediates the ATP-dependent assembly of spliceosomal U snRNPs","volume":"3","author":"Meister","year":"2001","journal-title":"Nat. Cell Biol."},{"key":"2021071913010577900_B38","doi-asserted-by":"crossref","first-page":"10045","DOI":"10.1073\/pnas.0802287105","article-title":"Evolution of an RNP assembly system: a minimal SMN complex facilitates formation of UsnRNPs in Drosophila melanogaster","volume":"105","author":"Kroiss","year":"2008","journal-title":"Proc. Natl. Acad. Sci. 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