{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T10:29:31Z","timestamp":1792146571629,"version":"4.4.0"},"reference-count":114,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,4,2]],"date-time":"2024-04-02T00:00:00Z","timestamp":1712016000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,4,2]],"date-time":"2024-04-02T00:00:00Z","timestamp":1712016000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Cell Commun Signal"],"abstract":"<jats:title>Abstract<\/jats:title><jats:p>This review presents a comprehensive exploration of the pivotal role played by the Linker of Nucleoskeleton and Cytoskeleton (LINC) complex, with a particular focus on Nesprin proteins, in cellular mechanics and the pathogenesis of muscular diseases. Distinguishing itself from prior works, the analysis delves deeply into the intricate interplay of the LINC complex, emphasizing its indispensable contribution to maintaining cellular structural integrity, especially in mechanically sensitive tissues such as cardiac and striated muscles. Additionally, the significant association between mutations in Nesprin proteins and the onset of Dilated Cardiomyopathy (DCM) and Emery-Dreifuss Muscular Dystrophy (EDMD) is highlighted, underscoring their pivotal role in disease pathogenesis. Through a comprehensive examination of DCM and EDMD cases, the review elucidates the disruptions in the LINC complex, nuclear morphology alterations, and muscular developmental disorders, thus emphasizing the essential function of an intact LINC complex in preserving muscle physiological functions. Moreover, the review provides novel insights into the implications of Nesprin mutations for cellular dynamics in the pathogenesis of muscular diseases, particularly in maintaining cardiac structural and functional integrity. Furthermore, advanced therapeutic strategies, including rectifying Nesprin gene mutations, controlling Nesprin protein expression, enhancing LINC complex functionality, and augmenting cardiac muscle cell function are proposed. By shedding light on the intricate molecular mechanisms underlying nuclear-cytoskeletal interactions, the review lays the groundwork for future research and therapeutic interventions aimed at addressing genetic muscle disorders.<\/jats:p>","DOI":"10.1186\/s12964-024-01593-y","type":"journal-article","created":{"date-parts":[[2024,4,2]],"date-time":"2024-04-02T08:01:55Z","timestamp":1712044915000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Nesprin proteins: bridging nuclear envelope dynamics to muscular dysfunction"],"prefix":"10.1186","volume":"22","author":[{"given":"Zhou","family":"Zi-yi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qin","family":"Qin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhou","family":"Fei","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cao","family":"Cun-Yu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teng","family":"Lin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,4,2]]},"reference":[{"issue":"9","key":"1593_CR1","doi-asserted-by":"publisher","first-page":"874","DOI":"10.1016\/j.semcdb.2010.08.006","volume":"21","author":"M ALMONACID","year":"2010","unstructured":"Almonacid M. Mechanisms controlling division-plane positioning [J]. Semin Cell Dev Biol. 2010;21(9):874\u201380.","journal-title":"Semin Cell Dev Biol"},{"key":"1593_CR2","doi-asserted-by":"publisher","first-page":"263","DOI":"10.1146\/annurev-genom-121219-083616","volume":"21","author":"X WONG","year":"2020","unstructured":"WONG X, STEWART CL. The laminopathies and the insights they provide into the structural and functional organization of the nucleus [J]. Annu Rev Genomics Hum Genet. 2020;21:263\u201388.","journal-title":"Annu Rev Genomics Hum Genet"},{"issue":"4","key":"1593_CR3","doi-asserted-by":"publisher","first-page":"379","DOI":"10.1038\/nmeth.1904","volume":"9","author":"D STRICKLAND","year":"2012","unstructured":"Strickland D, Lin Y, Wagner E, et al. TULIPs: tunable, light-controlled interacting protein tags for cell biology [J]. Nat Methods. 2012;9(4):379\u201384.","journal-title":"Nat Methods"},{"issue":"2","key":"1593_CR4","doi-asserted-by":"publisher","first-page":"357","DOI":"10.1016\/j.cell.2006.12.030","volume":"128","author":"ME JANSON","year":"2007","unstructured":"Janson ME, Loughlin R, Loiodice I, et al. Crosslinkers and motors organize dynamic microtubules to form stable bipolar arrays in fission yeast [J]. Cell. 2007;128(2):357\u201368.","journal-title":"Cell"},{"issue":"12","key":"1593_CR5","doi-asserted-by":"publisher","first-page":"6228","DOI":"10.1073\/pnas.94.12.6228","volume":"94","author":"M HOLY T E, DOGTEROM","year":"1997","unstructured":"Holy T E, Dogterom M. Assembly and positioning of microtubule asters in microfabricated chambers [J]. Proc Natl Acad Sci U S A. 1997;94(12):6228\u201331.","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"6753","key":"1593_CR6","doi-asserted-by":"publisher","first-page":"613","DOI":"10.1038\/44183","volume":"401","author":"R LOISEL T P, BOUJEMAA","year":"1999","unstructured":"Loisel T P, Boujemaa R. Reconstitution of actin-based motility of Listeria and Shigella using pure proteins [J]. Nature. 1999;401(6753):613\u20136.","journal-title":"Nature"},{"issue":"22","key":"1593_CR7","doi-asserted-by":"publisher","first-page":"7847","DOI":"10.1073\/pnas.0502121102","volume":"102","author":"E VAN DER GUCHT J, PALUCH","year":"2005","unstructured":"Van Der Gucht J, Paluch E, Plastino J, et al. Stress release drives symmetry breaking for actin-based movement [J]. Proc Natl Acad Sci U S A. 2005;102(22):7847\u201352.","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"7","key":"1593_CR8","doi-asserted-by":"publisher","first-page":"2143","DOI":"10.1016\/j.bpj.2010.07.055","volume":"99","author":"KAPITEIN","year":"2010","unstructured":"Kapitein, L C, Schlager M A, Van Der Zwan W A, et al. Probing intracellular motor protein activity using an inducible cargo trafficking assay [J]. Biophys J. 2010;99(7):2143\u201352.","journal-title":"Biophys J"},{"issue":"9","key":"1593_CR9","doi-asserted-by":"publisher","first-page":"828","DOI":"10.1016\/j.cub.2013.03.068","volume":"23","author":"LIPKAJ KAPITEIN L C, VAN BERGEIJK P","year":"2013","unstructured":"Kapitein L C, Van Bergeijk P Lipkaj, et al. Myosin-V opposes microtubule-based cargo transport and drives directional motility on cortical actin [J]. Curr Biol. 2013;23(9):828\u201334.","journal-title":"Curr Biol"},{"issue":"4","key":"1593_CR10","doi-asserted-by":"publisher","first-page":"470","DOI":"10.1038\/ncb3131","volume":"17","author":"M ALMONACID","year":"2015","unstructured":"Almonacid M, Ahmed W W, Bussonnier M, et al. Active diffusion positions the nucleus in mouse oocytes [J]. Nat Cell Biol. 2015;17(4):470\u20139.","journal-title":"Nat Cell Biol"},{"issue":"3","key":"1593_CR11","doi-asserted-by":"publisher","first-page":"961","DOI":"10.1016\/j.biomaterials.2013.10.037","volume":"35","author":"Q LI","year":"2014","unstructured":"Li Q, Kumar A. The regulation of dynamic mechanical coupling between actin cytoskeleton and nucleus by matrix geometry [J]. Biomaterials. 2014;35(3):961\u20139.","journal-title":"Biomaterials"},{"issue":"2","key":"1593_CR12","doi-asserted-by":"publisher","first-page":"305","DOI":"10.1083\/jcb.201610042","volume":"216","author":"S CHO","year":"2017","unstructured":"Cho S, Irianto J, DIscher DE. Mechanosensing by the nucleus: from pathways to scaling relationships [J]. J Cell Biol. 2017;216(2):305\u201315.","journal-title":"J Cell Biol"},{"issue":"2","key":"1593_CR13","doi-asserted-by":"publisher","first-page":"124","DOI":"10.3390\/cells8020124","volume":"8","author":"M Hieda","year":"2019","unstructured":"Hieda M. Signal transduction across the nuclear envelope: role of the LINC complex in bidirectional signaling [J]. Cells. 2019;8(2):124.","journal-title":"Cells"},{"key":"1593_CR14","doi-asserted-by":"publisher","first-page":"68","DOI":"10.1186\/s13036-019-0197-9","volume":"13","author":"T BOUZID","year":"2019","unstructured":"Bouzid T, Kim E, Riehl B D, et al. The LINC complex, mechanotransduction, and mesenchymal stem cell function and fate [J]. J Biol Eng. 2019;13:68.","journal-title":"J Biol Eng"},{"issue":"3","key":"1593_CR15","doi-asserted-by":"publisher","first-page":"1331","DOI":"10.1042\/BST20221541","volume":"51","author":"Z DE SILVA S, FAN","year":"2023","unstructured":"De Silva S, Fan Z, kang B, et al. Nesprin-1: novel regulator of striated muscle nuclear positioning and mechanotransduction [J]. Biochem Soc Trans. 2023;51(3):1331\u201345.","journal-title":"Biochem Soc Trans"},{"issue":"2","key":"1593_CR16","doi-asserted-by":"publisher","first-page":"311","DOI":"10.1042\/BST20170149","volume":"46","author":"C ZHOU","year":"2018","unstructured":"Zhou C, Rao L, Shanahan C M, et al. Nesprin-1\/2: roles in nuclear envelope organisation, myogenesis and muscle disease [J]. Biochem Soc Trans. 2018;46(2):311\u201320.","journal-title":"Biochem Soc Trans"},{"key":"1593_CR17","doi-asserted-by":"publisher","first-page":"38063","DOI":"10.1038\/srep38063","volume":"6","author":"Q ALAM S G, ZHANG","year":"2016","unstructured":"Alam S G, Zhang Q, Prasad N, et al. The mammalian LINC complex regulates genome transcriptional responses to substrate rigidity [J]. Sci Rep. 2016;6:38063.","journal-title":"Sci Rep"},{"issue":"10","key":"1593_CR18","doi-asserted-by":"publisher","first-page":"1440","DOI":"10.1038\/cr.2012.126","volume":"22","author":"W WANG","year":"2012","unstructured":"Wang W, Shi Z. Structural insights into SUN-KASH complexes across the nuclear envelope [J]. Cell Res. 2012;22(10):1440\u201352.","journal-title":"Cell Res"},{"key":"1593_CR19","doi-asserted-by":"publisher","first-page":"1277","DOI":"10.3389\/fphys.2018.01277","volume":"9","author":"A JANIN","year":"2018","unstructured":"JANIN A. Nesprins and lamins in health and diseases of cardiac and skeletal muscles [J]. Front Physiol. 2018;9:1277.","journal-title":"Front Physiol"},{"issue":"14","key":"1593_CR20","doi-asserted-by":"publisher","first-page":"339","DOI":"10.1136\/vr.128.14.339-a","volume":"128","author":"MEWS A R","year":"1991","unstructured":"Mews A R. Ban on sow stalls and tethers [J]. Vet Rec. 1991;128(14):339.","journal-title":"Vet Rec"},{"issue":"10","key":"1593_CR21","doi-asserted-by":"publisher","first-page":"3738","DOI":"10.1128\/MCB.26.10.3738-3751.2006","volume":"26","author":"F HAQUE","year":"2006","unstructured":"Haque F, Lloyd D J, Smallwood D T, et al. SUN1 interacts with nuclear lamin A and cytoplasmic nesprins to provide a physical connection between the nuclear lamina and the cytoskeleton [J]. Mol Cell Biol. 2006;26(10):3738\u201351.","journal-title":"Mol Cell Biol"},{"issue":"Pt 15","key":"1593_CR22","doi-asserted-by":"publisher","first-page":"3419","DOI":"10.1242\/jcs.02471","volume":"118","author":"T PADMAKUMAR V C, LIBOTTE","year":"2005","unstructured":"Padmakumar V C, LibottE T, Lu W, et al. The inner nuclear membrane protein Sun1 mediates the anchorage of Nesprin-2 to the nuclear envelope [J]. J Cell Sci. 2005;118(Pt 15):3419\u201330.","journal-title":"J Cell Sci"},{"issue":"5592","key":"1593_CR23","doi-asserted-by":"publisher","first-page":"406","DOI":"10.1126\/science.1075119","volume":"298","author":"HANM STARR D A","year":"2002","unstructured":"Starr D A Hanm. Role of ANC-1 in tethering nuclei to the actin cytoskeleton [J]. Science. 2002;298(5592):406\u20139.","journal-title":"Science"},{"issue":"7","key":"1593_CR24","doi-asserted-by":"publisher","first-page":"423","DOI":"10.1002\/cm.21182","volume":"71","author":"SJ KING","year":"2014","unstructured":"King SJ, Nowak K, Suryavanshi N, et al. Nesprin-1 and nesprin-2 regulate endothelial cell shape and migration [J]. Cytoskeleton (Hoboken). 2014;71(7):423\u201334.","journal-title":"Cytoskeleton (Hoboken)"},{"issue":"23","key":"1593_CR25","doi-asserted-by":"publisher","first-page":"2816","DOI":"10.1093\/hmg\/ddm238","volume":"16","author":"Q ZHANG","year":"2007","unstructured":"Zhang Q, Bethmann C, Worth N F, et al. Nesprin-1 and \u2013\u20092 are involved in the pathogenesis of Emery Dreifuss muscular dystrophy and are critical for nuclear envelope integrity [J]. Hum Mol Genet. 2007;16(23):2816\u201333.","journal-title":"Hum Mol Genet"},{"issue":"2","key":"1593_CR26","doi-asserted-by":"publisher","first-page":"126","DOI":"10.1080\/19491034.2016.1267093","volume":"8","author":"K PFISTERER","year":"2017","unstructured":"Pfisterer K, Jayo A. Control of nuclear organization by F-actin binding proteins [J]. Nucleus. 2017;8(2):126\u201333.","journal-title":"Nucleus"},{"issue":"12","key":"1593_CR27","doi-asserted-by":"publisher","first-page":"2258","DOI":"10.1093\/hmg\/ddx116","volume":"26","author":"C ZHOU","year":"2017","unstructured":"Zhou C, Li C, Zhou B, et al. Novel nesprin-1 mutations associated with dilated cardiomyopathy cause nuclear envelope disruption and defects in myogenesis [J]. Hum Mol Genet. 2017;26(12):2258\u201376.","journal-title":"Hum Mol Genet"},{"issue":"20","key":"1593_CR28","doi-asserted-by":"publisher","first-page":"15497","DOI":"10.3390\/ijms242015497","volume":"24","author":"GE Lee","year":"2023","unstructured":"Lee GE, Lee C, Byun J. Molecular mechanisms for the regulation of Nuclear membrane Integrity [J]. Int J Mol Sci. 2023;24(20):15497.","journal-title":"Int J Mol Sci"},{"key":"1593_CR29","doi-asserted-by":"publisher","first-page":"896","DOI":"10.3389\/fphys.2019.00896","volume":"10","author":"F Donnaloja","year":"2019","unstructured":"Donnaloja F, Jacchetti E, Soncimi M, et al. Mechanosensing at the Nuclear envelope by nuclear pore complex stretch activation and its effect in physiology and pathology [J]. Front Physiol. 2019;10:896.","journal-title":"Front Physiol."},{"issue":"4","key":"1593_CR30","doi-asserted-by":"publisher","first-page":"800","DOI":"10.1016\/j.cell.2020.03.052","volume":"181","author":"MM Nava","year":"2020","unstructured":"Nava MM, Miroshnikova YA, Biggs LC, et al. Heterochromatin-Driven Nuclear Softening protects the genome against mechanical stress-Induced damage [J]. Cell. 2020;181(4):800-17 e22.","journal-title":"Cell"},{"issue":"3","key":"1593_CR31","doi-asserted-by":"publisher","first-page":"669","DOI":"10.1042\/BST20180085","volume":"46","author":"C Zhou","year":"2018","unstructured":"Zhou C, Rao L, Warren DT, et al. Mouse models of nesprin-related diseases [J]. Biochem Soc Trans. 2018;46(3):669\u201381.","journal-title":"Biochem Soc Trans."},{"issue":"4","key":"1593_CR32","doi-asserted-by":"publisher","first-page":"607","DOI":"10.1093\/hmg\/ddn386","volume":"18","author":"MJ Puckelwartz","year":"2009","unstructured":"Puckelwartz MJ, Zhang Y, Kessler E. Disruption of nesprin-1 produces an Emery Dreifuss muscular dystrophy-like phenotype in mice [J]. Hum Mol Genet. 2009;18(4):607\u201320.","journal-title":"Hum Mol Genet."},{"issue":"17","key":"1593_CR33","doi-asserted-by":"publisher","first-page":"e2121816119","DOI":"10.1073\/pnas.2121816119","volume":"119","author":"A VAHABIKASHI","year":"2022","unstructured":"VahabikashI A, Sivagurunathan S, Nicdao F A S, et al. Nuclear lamin isoforms differentially contribute to LINC complex-dependent nucleocytoskeletal coupling and whole-cell mechanics [J]. Proc Natl Acad Sci U S A. 2022;119(17):e2121816119.","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"6","key":"1593_CR34","doi-asserted-by":"publisher","first-page":"1395","DOI":"10.3390\/cells9061395","volume":"9","author":"R Becker","year":"2020","unstructured":"Becker R, Leone M, Engel FB. Microtubule organization in striated muscle cells [J]. Cells. 2020;9(6):1395.","journal-title":"Cells"},{"issue":"3","key":"1593_CR35","doi-asserted-by":"publisher","first-page":"e10","DOI":"10.1161\/CIRCRESAHA.119.315582","volume":"126","author":"J Heffler","year":"2020","unstructured":"Heffler J, Shah PP, Robison P, et al. A Balance between Intermediate filaments and microtubules maintains nuclear architecture in the cardiomyocyte [J]. Circ Res. 2020;126(3):e10-26.","journal-title":"Circ Res."},{"key":"1593_CR36","doi-asserted-by":"publisher","first-page":"1126111","DOI":"10.3389\/fphys.2023.1126111","volume":"14","author":"B Zhang","year":"2023","unstructured":"Zhang B, Powers JD, Mcculloch AD, et al. Nuclear mechanosignaling in striated muscle diseases [J]. Front Physiol. 2023;14:1126111.","journal-title":"Front Physiol."},{"issue":"7","key":"1593_CR37","doi-asserted-by":"publisher","first-page":"1915","DOI":"10.1083\/jcb.201612128","volume":"216","author":"MJ Stroud","year":"2017","unstructured":"Stroud MJ, Feng W, Zhang J, et al. Nesprin 1\u03b12 is essential for mouse postnatal viability and nuclear positioning in skeletal muscle [J]. J Cell Biol. 2017;216(7):1915\u201324.","journal-title":"J Cell Biol."},{"issue":"1","key":"1593_CR38","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1083\/jcb.200509124","volume":"172","author":"M CRISP","year":"2006","unstructured":"Crisp M, Liu Q, Roux K, et al. Coupling of the nucleus and cytoplasm: role of the LINC complex [J]. J Cell Biol. 2006;172(1):41\u201353.","journal-title":"J Cell Biol"},{"issue":"30","key":"1593_CR39","doi-asserted-by":"publisher","first-page":"26743","DOI":"10.1074\/jbc.M111.233700","volume":"286","author":"ML LOMBARDI","year":"2011","unstructured":"Lombardi ML, Jaalouk D E, Shanahan C M, et al. The interaction between nesprins and sun proteins at the nuclear envelope is critical for force transmission between the nucleus and cytoskeleton [J]. J Biol Chem. 2011;286(30):26743\u201353.","journal-title":"J Biol Chem"},{"issue":"11","key":"1593_CR40","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1017\/S1462399405009294","volume":"7","author":"Q WARREN D T, ZHANG","year":"2005","unstructured":"Warren D T, Zhang Q, Weissberg P L, et al. Nesprins: intracellular scaffolds that maintain cell architecture and coordinate cell function? [J]. Expert Rev Mol Med. 2005;7(11):1\u201315.","journal-title":"Expert Rev Mol Med"},{"issue":"1","key":"1593_CR41","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1186\/s12881-017-0424-5","volume":"18","author":"Z CHEN","year":"2017","unstructured":"Chen Z, Ren Z, Mei W, et al. A novel SYNE1 gene mutation in a Chinese family of Emery-Dreifuss muscular dystrophy-like [J]. BMC Med Genet. 2017;18(1):63.","journal-title":"BMC Med Genet"},{"issue":"41","key":"1593_CR42","doi-asserted-by":"publisher","first-page":"31986","DOI":"10.1074\/jbc.M004775200","volume":"275","author":"RM APEL E D, LEWIS R M, GRADY","year":"2000","unstructured":"Apel E D, Lewis R M, Grady RM, et al. Syne-1, a dystrophin- and klarsicht-related protein associated with synaptic nuclei at the neuromuscular junction [J]. J Biol Chem. 2000;275(41):31986\u201395.","journal-title":"J Biol Chem"},{"issue":"1","key":"1593_CR43","doi-asserted-by":"publisher","first-page":"193","DOI":"10.1161\/01.RES.73.1.193","volume":"73","author":"JC SHANAHAN C M, WEISSBERG P L, METCAL","year":"1993","unstructured":"Shanahan C M, Weissberg P L, Metcalfe JC. Isolation of gene markers of differentiated and proliferating vascular smooth muscle cells [J]. Circ Res. 1993;73(1):193\u2013204.","journal-title":"Circ Res"},{"issue":"2","key":"1593_CR44","doi-asserted-by":"publisher","first-page":"682","DOI":"10.4049\/jimmunol.153.2.682","volume":"153","author":"J BEHRENS T W, JAGADEESH","year":"1994","unstructured":"Behrens T W, Jagadeesh J, Scherle P, et al. Jaw1, a lymphoid-restricted membrane protein localized to the endoplasmic reticulum [J]. J Immunol. 1994;153(2):682\u201390.","journal-title":"J Immunol"},{"issue":"Pt 24","key":"1593_CR45","doi-asserted-by":"publisher","first-page":"4485","DOI":"10.1242\/jcs.114.24.4485","volume":"114","author":"Q ZHANG","year":"2001","unstructured":"Zhang Q, Skepper J N, Yang F, et al. Nesprins: a novel family of spectrin-repeat-containing proteins that localize to the nuclear membrane in multiple tissues [J]. J Cell Sci. 2001;114(Pt 24):4485\u201398.","journal-title":"J Cell Sci"},{"issue":"5","key":"1593_CR46","doi-asserted-by":"publisher","first-page":"799","DOI":"10.1083\/jcb.200506083","volume":"171","author":"K WILHELMSEN","year":"2005","unstructured":"Wilhelmsen K, Litjens S H, Kuikman I, et al. Nesprin-3, a novel outer nuclear membrane protein, associates with the cytoskeletal linker protein plectin [J]. J Cell Biol. 2005;171(5):799\u2013810.","journal-title":"J Cell Biol"},{"issue":"2","key":"1593_CR47","doi-asserted-by":"publisher","first-page":"330","DOI":"10.1016\/j.yexcr.2004.01.014","volume":"295","author":"PADMAKUMARVC ABRAHAM","year":"2004","unstructured":"Abraham Padmakumarvc, Braune S. Enaptin, a giant actin-binding protein, is an element of the nuclear membrane and the actin cytoskeleton [J]. Exp Cell Res. 2004;295(2):330\u20139.","journal-title":"Exp Cell Res"},{"issue":"5","key":"1593_CR48","doi-asserted-by":"publisher","first-page":"028","DOI":"10.1093\/molehr\/gaab028","volume":"27","author":"D Zhang","year":"2021","unstructured":"Zhang D, Huang WJ, Chen GY, et al. Pathogenesis of acephalic spermatozoa syndrome caused by SUN5 variant [J]. Mol Hum Reprod. 2021;27(5):028.","journal-title":"Mol Hum Reprod"},{"issue":"2","key":"1593_CR49","first-page":"783","volume":"40","author":"Y Ji","year":"2018","unstructured":"Ji Y, Jiang J, Huang L, et al. Sperm\u2013associated antigen 4 (SPAG4) as a new cancer marker interacts with Nesprin3 to regulate cell migration in lung carcinoma [J]. Oncol Rep. 2018;40(2):783\u201392.","journal-title":"Oncol Rep."},{"issue":"7","key":"1593_CR50","doi-asserted-by":"publisher","first-page":"2194","DOI":"10.1073\/pnas.0808602106","volume":"106","author":"KJ Roux","year":"2009","unstructured":"Roux KJ, Crisp ML, Liu Q, et al. Nesprin 4 is an outer nuclear membrane protein that can induce kinesin-mediated cell polarization [J]. Proc Natl Acad Sci U S A. 2009;106(7):2194\u20139.","journal-title":"Proc Natl Acad Sci U S A."},{"key":"1593_CR51","doi-asserted-by":"publisher","first-page":"974168","DOI":"10.3389\/fcell.2022.974168","volume":"10","author":"S Taiber","year":"2022","unstructured":"Taiber S, Gozlan O, Cohen R, et al. A Nesprin-4\/kinesin-1 cargo model for nuclear positioning in cochlear outer hair cells [J]. Front Cell Dev Biol. 2022;10;974168.","journal-title":"Front Cell Dev Biol."},{"issue":"2","key":"1593_CR52","doi-asserted-by":"publisher","first-page":"e13259","DOI":"10.15252\/emmm.202013259","volume":"13","author":"S Taiber","year":"2021","unstructured":"Taiber S, Cohen R, Yizhar-Barnea O, et al. Neonatal AAV gene therapy rescues hearing in a mouse model of SYNE4 deafness [J]. EMBO Mol Med. 2021;13(2);e13259.","journal-title":"EMBO Mol Med."},{"issue":"2","key":"1593_CR53","first-page":"740","volume":"123","author":"HF HORN","year":"2013","unstructured":"Horn HF, Brownstein Z, Lenz D R, et al. The LINC complex is essential for hearing [J]. J Clin Invest. 2013;123(2):740\u201350.","journal-title":"J Clin Invest"},{"key":"1593_CR54","doi-asserted-by":"publisher","first-page":"e78201","DOI":"10.7554\/eLife.78201","volume":"11","author":"R Agrawal","year":"2022","unstructured":"Agrawal R, Gillies JP, Zang JL, et al. The KASH5 protein involved in meiotic chromosomal movements is a novel dynein activating adaptor [J]. Elife. 2022;11;e78201.","journal-title":"Elife"},{"issue":"15","key":"1593_CR55","doi-asserted-by":"publisher","first-page":"3207","DOI":"10.1242\/jcs.115.15.3207","volume":"115","author":"YY Zhen","year":"2002","unstructured":"Zhen YY, Libotte T, Munck M, et al. NUANCE, a giant protein connecting the nucleus and actin cytoskeleton [J]. J Cell Sci. 2002;115(15):3207\u201322.","journal-title":"J Cell Sci."},{"issue":"Pt 4","key":"1593_CR56","doi-asserted-by":"publisher","first-page":"673","DOI":"10.1242\/jcs.01642","volume":"118","author":"Q Zhang","year":"2005","unstructured":"Zhang Q, Ragnauth CD, Skepper JN, et al. Nesprin-2 is a multi-isomeric protein that binds lamin and emerin at the nuclear envelope and forms a subcellular network in skeletal muscle [J]. J Cell Sci. 2005;118(Pt 4):673\u201387.","journal-title":"J Cell Sci."},{"key":"1593_CR57","doi-asserted-by":"publisher","first-page":"221","DOI":"10.1007\/978-1-4939-3530-7_14","volume":"1411","author":"J CARTHEW","year":"2016","unstructured":"Carthew J. Detection of diverse and high Molecular Weight Nesprin-1 and Nesprin-2 isoforms using western blotting [J]. Methods Mol Biol. 2016;1411:221\u201332.","journal-title":"Methods Mol Biol"},{"issue":"20","key":"1593_CR58","doi-asserted-by":"publisher","first-page":"11289","DOI":"10.3390\/ijms222011289","volume":"22","author":"AG Liddane","year":"2021","unstructured":"Liddane AG, Holaska JM. The role of Emerin in cancer progression and metastasis [J]. Int J Mol Sci. 2021;22(20):11289.","journal-title":"Int J Mol Sci"},{"issue":"6","key":"1593_CR59","doi-asserted-by":"publisher","first-page":"1463","DOI":"10.3390\/cells9061463","volume":"9","author":"A Iyer","year":"2020","unstructured":"Iyer A, Holaska JM. EDMD-Causing emerin mutant myogenic progenitors exhibit impaired differentiation using similar mechanisms [J]. Cells. 2020;9(6):1463.","journal-title":"Cells"},{"issue":"4","key":"1593_CR60","doi-asserted-by":"publisher","first-page":"e94380","DOI":"10.1371\/journal.pone.0094380","volume":"9","author":"NT Duong","year":"2014","unstructured":"Duong NT, Morris GE, Lam LET, et al. Nesprins: tissue-specific expression of epsilon and other short isoforms [J]. Plos One. 2014;9(4):e94380.","journal-title":"Plos One."},{"issue":"7","key":"1593_CR61","doi-asserted-by":"publisher","first-page":"e40098","DOI":"10.1371\/journal.pone.0040098","volume":"7","author":"D Rajgor","year":"2012","unstructured":"Rajgor D, Mellad JA, Autore F, et al. Multiple novel nesprin-1 and nesprin-2 variants act as versatile tissue-specific intracellular scaffolds [J]. Plos One. 2012;7(7):e40098.","journal-title":"Plos One."},{"issue":"5","key":"1593_CR62","doi-asserted-by":"publisher","first-page":"473","DOI":"10.1006\/geno.2002.6859","volume":"80","author":"Q Zhang","year":"2002","unstructured":"Zhang Q, Ragnauth C, Greener MJ, et al. The nesprins are giant actin-binding proteins, orthologous to Drosophila melanogaster muscle protein MSP-300 [J]. Genomics. 2002;80(5):473\u201381.","journal-title":"Genomics"},{"issue":"6","key":"1593_CR63","doi-asserted-by":"publisher","first-page":"2410","DOI":"10.1091\/mbc.e02-07-0446","volume":"14","author":"GLL Fan","year":"2003","unstructured":"Fan GLL, Chu J. Golgi localization of Syne-1 [J]. Mol Biol Cell. 2003;14(6):2410\u201324.","journal-title":"Mol Biol Cell"},{"issue":"Pt 6","key":"1593_CR64","doi-asserted-by":"publisher","first-page":"1359","DOI":"10.1042\/BST0361359","volume":"36","author":"JG SIMPSON","year":"2008","unstructured":"Simpson JG, Roberts RG. Patterns of evolutionary conservation in the nesprin genes highlight probable functionally important protein domains and isoforms [J]. Biochem Soc Trans. 2008;36(Pt 6):1359\u201367.","journal-title":"Biochem Soc Trans"},{"issue":"5","key":"1593_CR65","doi-asserted-by":"publisher","first-page":"e63633","DOI":"10.1371\/journal.pone.0063633","volume":"8","author":"F AUTORE","year":"2013","unstructured":"Autore F, Pfuhl M, Quan X, et al. Large-scale modelling of the divergent spectrin repeats in nesprins: giant modular proteins [J]. PLoS ONE. 2013;8(5):e63633.","journal-title":"PLoS ONE"},{"issue":"1","key":"1593_CR66","doi-asserted-by":"publisher","first-page":"26","DOI":"10.1186\/s12860-016-0105-9","volume":"17","author":"Q HOLT I, DUONG N T, ZHANG","year":"2016","unstructured":"Holt I, Duong N T, Zhang Q, et al. Specific localization of nesprin-1-alpha2, the short isoform of nesprin-1 with a KASH domain, in developing, fetal and regenerating muscle, using a new monoclonal antibody [J]. BMC Cell Biol. 2016;17(1):26.","journal-title":"BMC Cell Biol"},{"issue":"1","key":"1593_CR67","doi-asserted-by":"publisher","first-page":"14202","DOI":"10.1038\/s41598-019-50728-6","volume":"9","author":"HOLT I","year":"2019","unstructured":"Holt I, Fuller H R, Lam L T, et al. Nesprin-1-alpha2 associates with kinesin at myotube outer nuclear membranes, but is restricted to neuromuscular junction nuclei in adult muscle [J]. Sci Rep. 2019;9(1):14202.","journal-title":"Sci Rep"},{"issue":"5","key":"1593_CR68","doi-asserted-by":"publisher","first-page":"913","DOI":"10.1083\/jcb.147.5.913","volume":"147","author":"T SULLIVAN","year":"1999","unstructured":"Sullivan T, Escalante-Alcalde D, Bhatt H, et al. Loss of A-type lamin expression compromises nuclear envelope integrity leading to muscular dystrophy [J]. J Cell Biol. 1999;147(5):913\u201320.","journal-title":"J Cell Biol"},{"issue":"2","key":"1593_CR69","doi-asserted-by":"publisher","first-page":"270","DOI":"10.1093\/eurheartj\/ehm342","volume":"29","author":"P ELLIOTT","year":"2008","unstructured":"Elliott P, Andersson B. Classification of the cardiomyopathies: a position statement from the European society of cardiology working group on myocardial and pericardial diseases [J]. Eur Heart J. 2008;29(2):270\u20136.","journal-title":"Eur Heart J"},{"issue":"3","key":"1593_CR70","doi-asserted-by":"publisher","first-page":"285","DOI":"10.1038\/6799","volume":"21","author":"G Bonne","year":"1999","unstructured":"Bonne G, Di Barletta MR, Varnous S, et al. Mutations in the gene encoding lamin A\/C cause autosomal dominant Emery-Dreifuss muscular dystrophy [J]. Nat Genet. 1999;21(3):285\u20138.","journal-title":"Nat Genet"},{"issue":"2","key":"1593_CR71","doi-asserted-by":"publisher","first-page":"285","DOI":"10.1016\/j.sbi.2013.03.005","volume":"23","author":"U SOSA B A, KUTAY","year":"2013","unstructured":"Sosa B A, Kutay U, Schwartz T U. Structural insights into LINC complexes [J]. Curr Opin Struct Biol. 2013;23(2):285\u201391.","journal-title":"Curr Opin Struct Biol"},{"issue":"6","key":"1593_CR72","doi-asserted-by":"publisher","first-page":"443","DOI":"10.1016\/j.nmd.2020.04.002","volume":"30","author":"EC STOREY","year":"2020","unstructured":"Storey EC, Holt I, Morris G E, et al. Muscle cell differentiation and development pathway defects in Emery-Dreifuss muscular dystrophy [J]. Neuromuscul Disord. 2020;30(6):443\u201356.","journal-title":"Neuromuscul Disord"},{"issue":"22","key":"1593_CR73","doi-asserted-by":"publisher","first-page":"1883","DOI":"10.1212\/01.wnl.0000263138.57257.6a","volume":"68","author":"R BEN YAOU","year":"2007","unstructured":"Ben Yaou R, Toutain A, Arimura T, et al. Multitissular involvement in a family with LMNA and EMD mutations: role of digenic mechanism? [J]. Neurology. 2007;68(22):1883\u201394.","journal-title":"Neurology"},{"issue":"4","key":"1593_CR74","doi-asserted-by":"publisher","first-page":"323","DOI":"10.1038\/ng1294-323","volume":"8","author":"S BIONE","year":"1994","unstructured":"Bione S, Maestrini E, Rivella S, et al. Identification of a novel X-linked gene responsible for Emery-Dreifuss muscular dystrophy [J]. Nat Genet. 1994;8(4):323\u20137.","journal-title":"Nat Genet"},{"issue":"12","key":"1593_CR75","doi-asserted-by":"publisher","first-page":"940","DOI":"10.1038\/nrg1906","volume":"7","author":"FS Collins","year":"2006","unstructured":"Collins FS, Capell BC. Human laminopathies: nuclei gone genetically awry [J]. Nat Rev Genet. 2006;7(12):940\u201352.","journal-title":"Nat Rev Genet"},{"issue":"3","key":"1593_CR76","doi-asserted-by":"publisher","first-page":"709","DOI":"10.1006\/bbrc.1999.2023","volume":"267","author":"L CLEMENTS","year":"2000","unstructured":"Clements L, Manilal S, Love D R, et al. Direct interaction between emerin and lamin A [J]. Biochem Biophys Res Commun. 2000;267(3):709\u201314.","journal-title":"Biochem Biophys Res Commun"},{"issue":"7","key":"1593_CR77","doi-asserted-by":"publisher","first-page":"832","DOI":"10.1101\/gad.1652708","volume":"22","author":"T DECHAT","year":"2008","unstructured":"Dechat T, Pfleghaar K, Sengupta K, et al. Nuclear lamins: major factors in the structural organization and function of the nucleus and chromatin [J]. Genes Dev. 2008;22(7):832\u201353.","journal-title":"Genes Dev"},{"issue":"3","key":"1593_CR78","doi-asserted-by":"publisher","first-page":"338","DOI":"10.1016\/j.ajhg.2009.07.015","volume":"85","author":"L GUENEAU","year":"2009","unstructured":"Gueneau L, Bertrand A T, Jais J P, et al. Mutations of the FHL1 gene cause Emery-Dreifuss muscular dystrophy [J]. Am J Hum Genet. 2009;85(3):338\u201353.","journal-title":"Am J Hum Genet"},{"issue":"5","key":"1593_CR79","doi-asserted-by":"publisher","first-page":"3487","DOI":"10.1074\/jbc.M109.071910","volume":"285","author":"F HAQUE","year":"2010","unstructured":"Haque F, Mazzeo D, Patel J T, et al. Mammalian SUN protein interaction networks at the inner nuclear membrane and their role in laminopathy disease processes [J]. J Biol Chem. 2010;285(5):3487\u201398.","journal-title":"J Biol Chem"},{"issue":"Pt 14","key":"1593_CR80","doi-asserted-by":"publisher","first-page":"3027","DOI":"10.1242\/jcs.00599","volume":"116","author":"I HOLT","year":"2003","unstructured":"Holt I, Ostlund C, Stewart C L, et al. Effect of pathogenic mis-sense mutations in lamin A on its interaction with emerin in vivo [J]. J Cell Sci. 2003;116(Pt 14):3027\u201335.","journal-title":"J Cell Sci"},{"issue":"24","key":"1593_CR81","doi-asserted-by":"publisher","first-page":"R1113","DOI":"10.1016\/j.cub.2013.11.009","volume":"23","author":"P ISERMANN","year":"2013","unstructured":"Isermann P, Lammerding J. Nuclear mechanics and mechanotransduction in health and disease [J]. Curr Biol. 2013;23(24):R1113\u201321.","journal-title":"Curr Biol"},{"issue":"5","key":"1593_CR82","doi-asserted-by":"publisher","first-page":"781","DOI":"10.1083\/jcb.200502148","volume":"170","author":"J LAMMERDING","year":"2005","unstructured":"Lammerding J, Hsiao J, Schulze P C, et al. Abnormal nuclear shape and impaired mechanotransduction in emerin-deficient cells [J]. J Cell Biol. 2005;170(5):781\u201391.","journal-title":"J Cell Biol"},{"issue":"6","key":"1593_CR83","doi-asserted-by":"publisher","first-page":"1005","DOI":"10.1002\/ana.22338","volume":"69","author":"H LIANG W C, MITSUHASHI","year":"2011","unstructured":"Liang W C, Mitsuhashi H. TMEM43 mutations in Emery-Dreifuss muscular dystrophy-related myopathy [J]. Ann Neurol. 2011;69(6):1005\u201313.","journal-title":"Ann Neurol"},{"issue":"14","key":"1593_CR84","doi-asserted-by":"publisher","first-page":"3275","DOI":"10.1038\/sj.emboj.7601230","volume":"25","author":"E MARKIEWICZ","year":"2006","unstructured":"Markiewicz E, Tilgner K, Barker N, et al. The inner nuclear membrane protein emerin regulates beta-catenin activity by restricting its accumulation in the nucleus [J]. EMBO J. 2006;25(14):3275\u201385.","journal-title":"EMBO J"},{"issue":"9","key":"1593_CR85","doi-asserted-by":"publisher","first-page":"e1004605","DOI":"10.1371\/journal.pgen.1004605","volume":"10","author":"P MEINKE","year":"2014","unstructured":"Meinke P, Mattioli E, Haque F, et al. Muscular dystrophy-associated SUN1 and SUN2 variants disrupt nuclear-cytoskeletal connections and myonuclear organization [J]. PLoS Genet. 2014;10(9):e1004605.","journal-title":"PLoS Genet"},{"issue":"1\u20133","key":"1593_CR86","doi-asserted-by":"publisher","first-page":"135","DOI":"10.1016\/S0014-5793(02)03105-8","volume":"525","author":"JM Mislow","year":"2002","unstructured":"Mislow JM, Holaska JM, Kim MS, et al. Nesprin-1alpha self-associates and binds directly to emerin and lamin A in vitro [J]. FEBS Lett. 2002;525(1\u20133):135\u201340.","journal-title":"FEBS Lett"},{"issue":"12","key":"1593_CR87","doi-asserted-by":"publisher","first-page":"4649","DOI":"10.1529\/biophysj.106.086454","volume":"91","author":"J ROWAT A C, LAMMERDING","year":"2006","unstructured":"Rowat A C, Lammerding J, Ipsen JH. Mechanical properties of the cell nucleus and the effect of emerin deficiency [J]. Biophys J. 2006;91(12):4649\u201364.","journal-title":"Biophys J"},{"key":"1593_CR88","doi-asserted-by":"publisher","first-page":"421","DOI":"10.1146\/annurev-cellbio-100109-104037","volume":"26","author":"STARR D A","year":"2010","unstructured":"Starr D A, Fridolfsson H N. Interactions between nuclei and the cytoskeleton are mediated by SUN-KASH nuclear-envelope bridges [J]. Annu Rev Cell Dev Biol. 2010;26:421\u201344.","journal-title":"Annu Rev Cell Dev Biol"},{"issue":"13","key":"1593_CR89","doi-asserted-by":"publisher","first-page":"2845","DOI":"10.1016\/j.yexcr.2007.03.025","volume":"313","author":"Q WHEELER M A, DAVIES J D, ZHANG","year":"2007","unstructured":"Wheeler M A, Davies J D, Zhang Q, et al. Distinct functional domains in nesprin-1\u03b1 and nesprin-2\u03b2 bind directly to emerin and both interactions are disrupted in X-linked Emery\u2013Dreifuss muscular dystrophy [J]. Exp Cell Res. 2007;313(13):2845\u201357.","journal-title":"Exp Cell Res"},{"issue":"4","key":"1593_CR90","doi-asserted-by":"publisher","first-page":"a000554","DOI":"10.1101\/cshperspect.a000554","volume":"2","author":"KL Wilson","year":"2010","unstructured":"Wilson KL, Foisner R. Lamin-binding proteins [J]. Cold Spring Harb Perspect Biol. 2010;2(4):a000554.","journal-title":"Cold Spring Harb Perspect Biol"},{"issue":"8","key":"1593_CR91","doi-asserted-by":"publisher","first-page":"499","DOI":"10.1016\/j.nmd.2010.05.010","volume":"20","author":"P SOLTANZADEH","year":"2010","unstructured":"Soltanzadeh P, Friez M J. Clinical and genetic characterization of manifesting carriers of DMD mutations [J]. Neuromuscul Disord. 2010;20(8):499\u2013504.","journal-title":"Neuromuscul Disord"},{"issue":"4","key":"1593_CR92","doi-asserted-by":"publisher","first-page":"5065","DOI":"10.3892\/mmr.2015.4065","volume":"12","author":"L ZHANG","year":"2015","unstructured":"Zhang L, Shen H, Zhao Z, et al. Cardiac effects of the c.1583 C\u2013>G LMNA mutation in two families with Emery-Dreifuss muscular dystrophy [J]. Mol Med Rep. 2015;12(4):5065\u201371.","journal-title":"Mol Med Rep"},{"issue":"1","key":"1593_CR93","doi-asserted-by":"publisher","first-page":"79","DOI":"10.1007\/s00109-004-0589-1","volume":"83","author":"VAN BERLO J H","year":"2005","unstructured":"Van Berlo J H, De Voogt W G, Van Der Kooi A J, et al. Meta-analysis of clinical characteristics of 299 carriers of LMNA gene mutations: do lamin A\/C mutations portend a high risk of sudden death? [J]. J Mol Med (Berl). 2005;83(1):79\u201383.","journal-title":"J Mol Med (Berl)"},{"issue":"1","key":"1593_CR94","doi-asserted-by":"publisher","first-page":"690","DOI":"10.1038\/s41467-021-20987-x","volume":"12","author":"Y RAMIREZ-MARTINEZ A, ZHANG","year":"2021","unstructured":"Ramirez-Martinez A, Zhang Y, Chen K, et al. The nuclear envelope protein Net39 is essential for muscle nuclear integrity and chromatin organization [J]. Nat Commun. 2021;12(1):690.","journal-title":"Nat Commun"},{"issue":"1","key":"1593_CR95","doi-asserted-by":"publisher","first-page":"145","DOI":"10.1002\/mus.24357","volume":"51","author":"M FANIN","year":"2015","unstructured":"Fanin M, Savarese M, Nascimbeni A C, et al. Dominant muscular dystrophy with a novel SYNE1 gene mutation [J]. Muscle Nerve. 2015;51(1):145\u20137.","journal-title":"Muscle Nerve"},{"key":"1593_CR96","doi-asserted-by":"publisher","first-page":"690577","DOI":"10.3389\/fcell.2021.690577","volume":"9","author":"MJA Van Ingen","year":"2021","unstructured":"Van Ingen MJA, Kirby TJ. LINCing nuclear mechanobiology with skeletal muscle mass and function [J]. Front Cell Dev Biol. 2021;9;690577.","journal-title":"Front Cell Dev Biol"},{"issue":"1","key":"1593_CR97","doi-asserted-by":"publisher","first-page":"218","DOI":"10.1242\/dev.114769","volume":"142","author":"MH WILSON","year":"2015","unstructured":"Wilson MH, Holzbaur EL. Nesprins Anchor kinesin-1 motors to the nucleus to drive nuclear distribution in muscle cells [J]. Development. 2015;142(1):218\u201328.","journal-title":"Development"},{"issue":"19","key":"1593_CR98","doi-asserted-by":"publisher","first-page":"2999","DOI":"10.1016\/j.cub.2017.08.031","volume":"27","author":"P GIMPEL","year":"2017","unstructured":"Gimpel P, Lee Y L, Sobota R M, et al. Nesprin-1alpha-Dependent microtubule nucleation from the Nuclear envelope via Akap450 is necessary for nuclear positioning in muscle cells [J]. Curr Biol. 2017;27(19):2999\u20133009. e9.","journal-title":"Curr Biol"},{"issue":"4","key":"1593_CR99","doi-asserted-by":"publisher","first-page":"600","DOI":"10.1016\/j.yjmcc.2009.11.006","volume":"48","author":"MJ PUCKELWARTZ","year":"2010","unstructured":"Puckelwartz MJ, Kessler E J, Kim G, et al. Nesprin-1 mutations in human and murine cardiomyopathy [J]. J Mol Cell Cardiol. 2010;48(4):600\u20138.","journal-title":"J Mol Cell Cardiol"},{"issue":"4","key":"1593_CR100","doi-asserted-by":"publisher","first-page":"452","DOI":"10.1002\/humu.22504","volume":"35","author":"P LI","year":"2014","unstructured":"Li P, Meinke P, Huong L T T, et al. Contribution of SUN1 mutations to the pathomechanism in muscular dystrophies [J]. Hum Mutat. 2014;35(4):452\u201361.","journal-title":"Hum Mutat"},{"issue":"24","key":"1593_CR101","doi-asserted-by":"publisher","first-page":"4447","DOI":"10.1242\/jcs.114.24.4447","volume":"114","author":"P RAHARJO W H, ENARSON","year":"2001","unstructured":"Raharjo W H, Enarson P, Sullivan T, et al. Nuclear envelope defects associated withLMNAmutations cause dilated cardiomyopathy and Emery-Dreifuss muscular dystrophy [J]. J Cell Sci. 2001;114(24):4447\u201357.","journal-title":"J Cell Sci"},{"issue":"2","key":"1593_CR102","doi-asserted-by":"publisher","first-page":"e1004114","DOI":"10.1371\/journal.pgen.1004114","volume":"10","author":"DZ Wang","year":"2014","unstructured":"Wang DZ, Banerjee I, Zhang J, et al. Targeted ablation of Nesprin 1 and Nesprin 2 from murine myocardium results in cardiomyopathy, altered Nuclear morphology and inhibition of the Biomechanical Gene response [J]. PLoS Genet. 2014;10(2):e1004114.","journal-title":"PLoS Genet"},{"issue":"15","key":"1593_CR103","doi-asserted-by":"publisher","first-page":"1333","DOI":"10.1177\/1535370219862243","volume":"244","author":"C \u00d6STLUND","year":"2019","unstructured":"\u00d6stlund C, Chang W, Gundersen G G, et al. Pathogenic mutations in genes encoding nuclear envelope proteins and defective nucleocytoplasmic connections [J]. Experimental Biology Med. 2019;244(15):1333\u201344.","journal-title":"Experimental Biology Med"},{"issue":"2","key":"1593_CR104","doi-asserted-by":"publisher","first-page":"177","DOI":"10.1093\/hmg\/ddac179","volume":"32","author":"E L Leong","year":"2023","unstructured":"Leong E L, Khaing N T, Cadot B, et al. Nesprin-1 LINC complexes recruit microtubule cytoskeleton proteins and drive pathology in Lmna-mutant striated muscle [J]. Hum Mol Genet. 2023;32(2):177\u201391.","journal-title":"Hum Mol Genet"},{"issue":"6","key":"1593_CR105","doi-asserted-by":"publisher","first-page":"1602","DOI":"10.1053\/j.gastro.2018.03.026","volume":"154","author":"G F BRADY","year":"2018","unstructured":"BRADY G F, Kwan R, Bragazzi Cunha J, et al. Lamins and Lamin-Associated Proteins in Gastrointestinal Health and Disease [J]. Gastroenterology. 2018;154(6):1602-e191.","journal-title":"Gastroenterology"},{"issue":"1","key":"1593_CR106","doi-asserted-by":"publisher","first-page":"322","DOI":"10.1016\/j.bbagen.2013.09.032","volume":"1840","author":"JL Ligget","year":"2014","unstructured":"Ligget JL, Choi CK, Donnell RL, et al. Nonsteroidal anti-inflammatory drug sulindac sulfide suppresses structural protein Nesprin-2 expression in colorectal cancer cells [J]. Biochimica et Biophysica Acta (BBA) - Gen Subj. 2014;1840(1):322\u201331.","journal-title":"Biochimica et Biophysica Acta (BBA) - Gen Subj"},{"issue":"2","key":"1593_CR107","doi-asserted-by":"publisher","first-page":"173","DOI":"10.4161\/nucl.29023","volume":"5","author":"I Sur","year":"2014","unstructured":"Sur I, Neumann S, Noegel AA. Nesprin-1 role in DNA damage response [J]. Nucleus. 2014;5(2):173\u201391.","journal-title":"Nucleus."},{"issue":"11","key":"1593_CR108","doi-asserted-by":"publisher","first-page":"3061","DOI":"10.3390\/cells10113061","volume":"10","author":"A Rey","year":"2021","unstructured":"Rey A, Schaeffer L, Durand B. Drosophila Nesprin-1 isoforms differentially contribute to muscle function [J]. Cells. 2021;10(11):3061.","journal-title":"Cells"},{"issue":"10","key":"1593_CR109","doi-asserted-by":"publisher","first-page":"346","DOI":"10.1111\/boc.201400033","volume":"106","author":"M CARMOSINO","year":"2014","unstructured":"Carmosino M, Torretta S, Procino G, et al. Role of nuclear lamin A\/C in cardiomyocyte functions [J]. Biol Cell. 2014;106(10):346\u201358.","journal-title":"Biol Cell"},{"issue":"10","key":"1593_CR110","doi-asserted-by":"publisher","first-page":"2121","DOI":"10.1016\/j.yexcr.2007.03.028","volume":"313","author":"H J Worman","year":"2007","unstructured":"Worman H J, Bonne G. Laminopathies: a wide spectrum of human diseases [J]. Exp Cell Res. 2007;313(10):2121\u201333.","journal-title":"Exp Cell Res"},{"issue":"1","key":"1593_CR111","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1038\/nrm3488","volume":"14","author":"B BURKE","year":"2013","unstructured":"Burke B, Stewart CL. The nuclear lamins: flexibility in function [J]. Nat Rev Mol Cell Biol. 2013;14(1):13\u201324.","journal-title":"Nat Rev Mol Cell Biol"},{"issue":"1","key":"1593_CR112","doi-asserted-by":"publisher","first-page":"8738","DOI":"10.1038\/s41598-020-65793-5","volume":"10","author":"M Bostani","year":"2020","unstructured":"Bostani M, Rahmati M, Mard SA. The effect of endurance training on levels of LINC complex proteins in skeletal muscle fibers of STZ-induced diabetic rats [J]. Sci Rep. 2020;10(1):8738.","journal-title":"Sci Rep"},{"key":"1593_CR113","doi-asserted-by":"publisher","first-page":"4","DOI":"10.1038\/s41419-022-04729-5","volume":"13","author":"F Chiarini","year":"2022","unstructured":"Chiarini F, Paganelli F, Balestra T, et al. Lamin A and the LINC complex act as potential tumor suppressors in Ewing Sarcoma [J]. Cell Death Dis. 2022;13:4.","journal-title":"Cell Death Dis"},{"issue":"25","key":"1593_CR114","doi-asserted-by":"publisher","first-page":"2768","DOI":"10.1091\/mbc.E20-02-0127","volume":"31","author":"P COL\u00f3N-BOLEA","year":"2020","unstructured":"Col\u00f3n-Bolea P, Garc\u00eda-G\u00f3mez R, Shackleton S, et al. RAC1 induces nuclear alterations through the LINC complex to enhance melanoma invasiveness [J]. Mol Biol Cell. 2020;31(25):2768\u201378.","journal-title":"Mol Biol Cell"}],"container-title":["Cell Communication and Signaling"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12964-024-01593-y.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s12964-024-01593-y\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12964-024-01593-y.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,4,2]],"date-time":"2024-04-02T09:12:12Z","timestamp":1712049132000},"score":1,"resource":{"primary":{"URL":"https:\/\/biosignaling.biomedcentral.com\/articles\/10.1186\/s12964-024-01593-y"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,4,2]]},"references-count":114,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["1593"],"URL":"https:\/\/doi.org\/10.1186\/s12964-024-01593-y","relation":{},"ISSN":["1478-811X"],"issn-type":[{"value":"1478-811X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,4,2]]},"assertion":[{"value":"28 January 2024","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"24 March 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"2 April 2024","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare no competing interests.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"208"}}