{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T18:21:52Z","timestamp":1787768512069,"version":"build-2784847793"},"reference-count":73,"publisher":"American Association for the Advancement of Science (AAAS)","issue":"35","funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R35GM134859"],"award-info":[{"award-number":["R35GM134859"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100017023","name":"Paul G. Allen Frontiers Group","doi-asserted-by":"publisher","award":["Allen Distinguished Investigator Award"],"award-info":[{"award-number":["Allen Distinguished Investigator Award"]}],"id":[{"id":"10.13039\/100017023","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100017023","name":"Paul G. Allen Frontiers Group","doi-asserted-by":"publisher","award":["Allen Distinguished Investigator Award"],"award-info":[{"award-number":["Allen Distinguished Investigator Award"]}],"id":[{"id":"10.13039\/100017023","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["www.science.org"],"crossmark-restriction":true},"short-container-title":["Sci. Adv."],"published-print":{"date-parts":[[2026,8,28]]},"abstract":"<jats:p>\n                    Emery-Dreifuss muscular dystrophy (EDMD) arises from mutations in nuclear lamins or emerin. Current pathological models emphasize defective nuclear mechanics and transcriptional regulation, yet these mechanisms cannot explain how lamina defects propagate across the cell to produce the complex pathology of laminopathies. Here, we reveal an emerging pathway linking nuclear lamina dysfunction to cytoplasmic reorganization. Using\n                    <jats:italic toggle=\"yes\">Caenorhabditis elegans<\/jats:italic>\n                    EDMD models, we show that disease-linked lamin variants reduce cytoplasmic mesoscale crowding, increase molecular diffusivity, and disrupt nuclear positioning and endoplasmic reticulum architecture, which mirror phenotypes caused by ribosome depletion. Lamin dysfunction also lowers nucleolar fibrillarin levels and ribosome abundance, revealing a nucleolar-ribosomal axis that transmits nuclear defects to the cytoplasm. Loss of the redundant LEM-domain proteins\n                    <jats:italic toggle=\"yes\">emr-1<\/jats:italic>\n                    and\n                    <jats:italic toggle=\"yes\">lem-2<\/jats:italic>\n                    phenocopied lamin mutants, indicating that cytoplasmic disorganization is a shared hallmark of EDMD. These findings connect nuclear architecture to whole-cell biophysics and suggest therapeutic strategies aimed at restoring ribosome function.\n                  <\/jats:p>","DOI":"10.1126\/sciadv.aec9028","type":"journal-article","created":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T17:59:39Z","timestamp":1787767179000},"update-policy":"https:\/\/doi.org\/10.34133\/aaas_crossmark","source":"Crossref","is-referenced-by-count":0,"title":["Muscular dystrophy\u2013associated lamin variants disrupt cellular organization through a nucleolar-ribosomal axis"],"prefix":"10.1126","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-4831-1274","authenticated-orcid":true,"given":"Xiangyi","family":"Ding","sequence":"first","affiliation":[{"name":"Department of Molecular and Cellular Biology, University of California, Davis, Davis, CA, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sweta","family":"Kumari","sequence":"additional","affiliation":[{"name":"Department of Molecular and Cellular Biology, University of California, Davis, Davis, CA, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2228-8258","authenticated-orcid":true,"given":"Ellen","family":"F. Gregory","sequence":"additional","affiliation":[{"name":"Department of Molecular and Cellular Biology, University of California, Davis, Davis, CA, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7339-6606","authenticated-orcid":true,"given":"Daniel A.","family":"Starr","sequence":"additional","affiliation":[{"name":"Department of Molecular and Cellular Biology, University of California, Davis, Davis, CA, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6180-8906","authenticated-orcid":true,"given":"G. W. Gant","family":"Luxton","sequence":"additional","affiliation":[{"name":"Department of Molecular and Cellular Biology, University of California, Davis, Davis, CA, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"221","reference":[{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.26782"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ceb.2023.102313"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1042\/BST20110724"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ceb.2015.03.005"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.3389\/fphys.2013.00363"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddn386"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.e14-05-0971"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.26.10.3738-3751.2006"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.e16-10-0721"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-genom-121219-083616"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2018.05.042"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1126\/sciadv.adx0952"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.1021\/acs.chemrev.3c00615"},{"key":"e_1_3_2_15_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41580-020-0272-6"},{"key":"e_1_3_2_16_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0708974105"},{"key":"e_1_3_2_17_2","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.11.11.3937"},{"key":"e_1_3_2_18_2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1010895"},{"key":"e_1_3_2_19_2","doi-asserted-by":"publisher","DOI":"10.1895\/wormbook.1.89.1"},{"key":"e_1_3_2_20_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cub.2011.08.030"},{"key":"e_1_3_2_21_2","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.61069"},{"key":"e_1_3_2_22_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1075119"},{"key":"e_1_3_2_23_2","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4939-8691-0_13"},{"key":"e_1_3_2_24_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cub.2018.08.001"},{"key":"e_1_3_2_25_2","doi-asserted-by":"publisher","DOI":"10.3390\/cells10092341"},{"key":"e_1_3_2_26_2","article-title":"Perimetric complexity of binary digital images","volume":"14","author":"Watson A.","year":"2012","unstructured":"A. Watson, Perimetric complexity of binary digital images. Math. J. 14, 10.3888\/tmj.14-5 (2012).","journal-title":"Math. J."},{"key":"e_1_3_2_27_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-017-00322-z"},{"key":"e_1_3_2_28_2","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.046284"},{"key":"e_1_3_2_29_2","doi-asserted-by":"publisher","DOI":"10.1155\/2012\/601274"},{"key":"e_1_3_2_30_2","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.23.23.8519-8527.2003"},{"key":"e_1_3_2_31_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1017150108"},{"key":"e_1_3_2_32_2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0040290"},{"key":"e_1_3_2_33_2","doi-asserted-by":"crossref","unstructured":"E. L. Spaulding D. L. Updike C. elegans nucleolar RG repeats are sufficient for nucleolar accumulation but insufficient for sub-nucleolar compartmentalization. bioRxiv 2024.12.19.629445 [Preprint] (2024). https:\/\/doi.org\/10.1101\/2024.12.19.629445.","DOI":"10.1101\/2024.12.19.629445"},{"key":"e_1_3_2_34_2","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.26376"},{"key":"e_1_3_2_35_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1232044"},{"key":"e_1_3_2_36_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0730821100"},{"key":"e_1_3_2_37_2","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.e11-06-0505"},{"key":"e_1_3_2_38_2","doi-asserted-by":"publisher","DOI":"10.1534\/genetics.116.197160"},{"key":"e_1_3_2_39_2","doi-asserted-by":"publisher","DOI":"10.1242\/dev.126.14.3171"},{"key":"e_1_3_2_40_2","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.e05-09-0894"},{"key":"e_1_3_2_41_2","doi-asserted-by":"publisher","DOI":"10.1177\/1535370219871965"},{"key":"e_1_3_2_42_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddm238"},{"key":"e_1_3_2_43_2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1004605"},{"key":"e_1_3_2_44_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.dib.2017.05.028"},{"key":"e_1_3_2_45_2","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.00274-17"},{"key":"e_1_3_2_46_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1509317112"},{"key":"e_1_3_2_47_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.2411964122"},{"key":"e_1_3_2_48_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cub.2015.11.065"},{"key":"e_1_3_2_49_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-024-54120-5"},{"key":"e_1_3_2_50_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-025-60771-9"},{"key":"e_1_3_2_51_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41586-025-09207-4"},{"key":"e_1_3_2_52_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41556-024-01363-5"},{"key":"e_1_3_2_53_2","doi-asserted-by":"publisher","DOI":"10.3390\/cells8080869"},{"key":"e_1_3_2_54_2","doi-asserted-by":"publisher","DOI":"10.3390\/ijms26094174"},{"key":"e_1_3_2_55_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2025.116204"},{"key":"e_1_3_2_56_2","doi-asserted-by":"publisher","DOI":"10.1895\/wormbook.1.81.2"},{"key":"e_1_3_2_57_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.bpj.2011.02.035"},{"key":"e_1_3_2_58_2","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.184309"},{"key":"e_1_3_2_59_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.crmeth.2022.100203"},{"key":"e_1_3_2_60_2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1007520"},{"key":"e_1_3_2_61_2","doi-asserted-by":"publisher","DOI":"10.1080\/19491034.2016.1238999"},{"key":"e_1_3_2_62_2","doi-asserted-by":"publisher","DOI":"10.3389\/fphys.2018.01533"},{"key":"e_1_3_2_63_2","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.3003802"},{"key":"e_1_3_2_64_2","doi-asserted-by":"publisher","DOI":"10.1038\/sj.onc.1209883"},{"key":"e_1_3_2_65_2","doi-asserted-by":"publisher","DOI":"10.1186\/s12967-023-04170-y"},{"key":"e_1_3_2_66_2","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.00260-09"},{"key":"e_1_3_2_67_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2010.02.065"},{"key":"e_1_3_2_68_2","doi-asserted-by":"crossref","unstructured":"J. Odell Y. Tang Y. S. Ambekar G. R. Kidiyoor H. Saadi G. F. Woodworth L. J. Holt G. Scarcelli H. Yu J. Lammerding The Deformability of the Mammalian Cell Nucleus is Determined by the Identity of the Lamin Rod Domain. bioRxiv 2025.07.22.666133 [Preprint] (2025). https:\/\/doi.org\/10.1101\/2025.07.22.666133.","DOI":"10.1101\/2025.07.22.666133"},{"key":"e_1_3_2_69_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.2406340121"},{"key":"e_1_3_2_70_2","doi-asserted-by":"publisher","DOI":"10.1093\/genetics\/77.1.71"},{"key":"e_1_3_2_71_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1046-2023(03)00050-1"},{"key":"e_1_3_2_72_2","doi-asserted-by":"publisher","DOI":"10.1186\/gb-2000-2-1-research0002"},{"key":"e_1_3_2_73_2","doi-asserted-by":"publisher","DOI":"10.1534\/g3.114.013649"},{"key":"e_1_3_2_74_2","doi-asserted-by":"publisher","DOI":"10.1109\/TSMC.1979.4310076"}],"container-title":["Science Advances"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.science.org\/doi\/pdf\/10.1126\/sciadv.aec9028","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T18:01:32Z","timestamp":1787767292000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.science.org\/doi\/10.1126\/sciadv.aec9028"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8,28]]},"references-count":73,"journal-issue":{"issue":"35","published-print":{"date-parts":[[2026,8,28]]}},"alternative-id":["10.1126\/sciadv.aec9028"],"URL":"https:\/\/doi.org\/10.1126\/sciadv.aec9028","relation":{},"ISSN":["2375-2548"],"issn-type":[{"value":"2375-2548","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,8,28]]},"assertion":[{"value":"2025-10-07","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-07-15","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-08-26","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"eaec9028"}}