{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,24]],"date-time":"2025-10-24T16:45:09Z","timestamp":1761324309979,"version":"build-2065373602"},"reference-count":41,"publisher":"MDPI AG","issue":"17","license":[{"start":{"date-parts":[[2020,8,19]],"date-time":"2020-08-19T00:00:00Z","timestamp":1597795200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"SEP-CINVESTAV","award":["Reference 242"],"award-info":[{"award-number":["Reference 242"]}]},{"name":"Bilateral Agreement CNR (Italy)\/CINVESTAV (Mexico)","award":["CNR2018\/4"],"award-info":[{"award-number":["CNR2018\/4"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>\u03b2-dystroglycan (\u03b2-DG) assembles with lamins A\/C and B1 and emerin at the nuclear envelope (NE) to maintain proper nuclear architecture and function. To provide insight into the nuclear function of \u03b2-DG, we characterized the interaction between \u03b2-DG and emerin at the molecular level. Emerin is a major NE protein that regulates multiple nuclear processes and whose deficiency results in Emery\u2013Dreifuss muscular dystrophy (EDMD). Using truncated variants of \u03b2-DG and emerin, via a series of in vitro and in vivo binding experiments and a tailored computational analysis, we determined that the \u03b2-DG\u2013emerin interaction is mediated at least in part by their respective transmembrane domains (TM). Using surface plasmon resonance assays we showed that emerin binds to \u03b2-DG with high affinity (KD in the nanomolar range). Remarkably, the analysis of cells in which DG was knocked out demonstrated that loss of \u03b2-DG resulted in a decreased emerin stability and impairment of emerin-mediated processes. \u03b2-DG and emerin are reciprocally required for their optimal targeting within the NE, as shown by immunofluorescence, western blotting and immunoprecipitation assays using emerin variants with mutations in the TM domain and B-lymphocytes of a patient with EDMD. In summary, we demonstrated that \u03b2-DG plays a role as an emerin interacting partner modulating its stability and function.<\/jats:p>","DOI":"10.3390\/ijms21175944","type":"journal-article","created":{"date-parts":[[2020,8,19]],"date-time":"2020-08-19T09:22:31Z","timestamp":1597828951000},"page":"5944","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["The Molecular Basis and Biologic Significance of the \u03b2-Dystroglycan-Emerin Interaction"],"prefix":"10.3390","volume":"21","author":[{"given":"Wendy Lili\u00e1n","family":"G\u00f3mez-Monsivais","sequence":"first","affiliation":[{"name":"Department of Genetics and Molecular Biology, Centro de Investigaci\u00f3n y de Estudios Avanzados del Instituto Polit\u00e9cnico Nacional (CINVESTAV), 07360 Mexico City, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Feliciano","family":"Monterrubio-Ledezma","sequence":"additional","affiliation":[{"name":"Department of Genetics and Molecular Biology, Centro de Investigaci\u00f3n y de Estudios Avanzados del Instituto Polit\u00e9cnico Nacional (CINVESTAV), 07360 Mexico City, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jazmin","family":"Huerta-Cantillo","sequence":"additional","affiliation":[{"name":"Cell Biology Department, Centro de Investigaci\u00f3n y de Estudios Avanzados del Instituto Polit\u00e9cnico Nacional (CINVESTAV), 07360 Mexico City, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ricardo","family":"Mondragon-Gonzalez","sequence":"additional","affiliation":[{"name":"Department of Genetics and Molecular Biology, Centro de Investigaci\u00f3n y de Estudios Avanzados del Instituto Polit\u00e9cnico Nacional (CINVESTAV), 07360 Mexico City, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alma","family":"Alamillo-Iniesta","sequence":"additional","affiliation":[{"name":"Department of Genetics and Molecular Biology, Centro de Investigaci\u00f3n y de Estudios Avanzados del Instituto Polit\u00e9cnico Nacional (CINVESTAV), 07360 Mexico City, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ian","family":"Garc\u00eda-Aguirre","sequence":"additional","affiliation":[{"name":"Department of Genetics and Molecular Biology, Centro de Investigaci\u00f3n y de Estudios Avanzados del Instituto Polit\u00e9cnico Nacional (CINVESTAV), 07360 Mexico City, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paulina Margarita","family":"Azuara-Medina","sequence":"additional","affiliation":[{"name":"Department of Genetics and Molecular Biology, Centro de Investigaci\u00f3n y de Estudios Avanzados del Instituto Polit\u00e9cnico Nacional (CINVESTAV), 07360 Mexico City, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ra\u00fal","family":"Arguello-Garc\u00eda","sequence":"additional","affiliation":[{"name":"Department of Genetics and Molecular Biology, Centro de Investigaci\u00f3n y de Estudios Avanzados del Instituto Polit\u00e9cnico Nacional (CINVESTAV), 07360 Mexico City, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6911-9988","authenticated-orcid":false,"given":"Jhon Erick","family":"Rivera-Monroy","sequence":"additional","affiliation":[{"name":"Department of Molecular Biology, Faculty of Medicine, GZMB, Georg-August University, 37073 G\u00f6ttingen, Germany"},{"name":"Laboratorio Instrumental de Alta Complejidad, Universidad de La Salle, 111311 Bogot\u00e1, Colombia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0273-9995","authenticated-orcid":false,"given":"James M.","family":"Holaska","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences, Cooper Medical School of Rowan University, 401 S Broadway, Camden, NJ 08028, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7153-4964","authenticated-orcid":false,"given":"Jes\u00fas Mauricio Ernesto","family":"Hern\u00e1ndez-M\u00e9ndez","sequence":"additional","affiliation":[{"name":"Department of Genetics and Molecular Biology, Centro de Investigaci\u00f3n y de Estudios Avanzados del Instituto Polit\u00e9cnico Nacional (CINVESTAV), 07360 Mexico City, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Efra\u00edn","family":"Garrido","sequence":"additional","affiliation":[{"name":"Department of Genetics and Molecular Biology, Centro de Investigaci\u00f3n y de Estudios Avanzados del Instituto Polit\u00e9cnico Nacional (CINVESTAV), 07360 Mexico City, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4399-4618","authenticated-orcid":false,"given":"Jonathan Javier","family":"Maga\u00f1a","sequence":"additional","affiliation":[{"name":"Laboratory of Genomic Medicine, Department of Genetics, National Rehabilitation Institute-Luis Guillermo Ibarra Ibarra (INR-LGII), 14389 Mexico City, Mexico"},{"name":"Departamento de Bioingenier\u00eda, Escuela de Ingenier\u00eda y Ciencias, Instituto Tecnol\u00f3gico y de Estudios Superiores de Monterrey-Campus Ciudad de M\u00e9xico, 14380 Ciudad de M\u00e9xico, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Steve J.","family":"Winder","sequence":"additional","affiliation":[{"name":"Department of Biomedical Science, University of Sheffield, Western Bank, Sheffield S10 2TN, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4690-8826","authenticated-orcid":false,"given":"Andrea","family":"Brancaccio","sequence":"additional","affiliation":[{"name":"School of Biochemistry, University of Bristol, Bristol BS8 1TD, UK"},{"name":"Institute of Chemical Sciences and Technologies \u201cGiulio Natta\u201d (SCITEC), 00168 Roma, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ivette","family":"Mart\u00ednez-Vieyra","sequence":"additional","affiliation":[{"name":"Laboratory of Hematobiology, Escuela Nacional de Medicina y Homeopat\u00eda, Instituto Polit\u00e9cnico Nacional, 07320 Ciudad de M\u00e9xico, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fernando","family":"Navarro-Garcia","sequence":"additional","affiliation":[{"name":"Cell Biology Department, Centro de Investigaci\u00f3n y de Estudios Avanzados del Instituto Polit\u00e9cnico Nacional (CINVESTAV), 07360 Mexico City, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bulmaro","family":"Cisneros","sequence":"additional","affiliation":[{"name":"Department of Genetics and Molecular Biology, Centro de Investigaci\u00f3n y de Estudios Avanzados del Instituto Polit\u00e9cnico Nacional (CINVESTAV), 07360 Mexico City, Mexico"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2020,8,19]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1016\/j.ceb.2018.05.001","article-title":"Insight into the functional organization of nuclear lamins in health and disease","volume":"54","author":"Tatli","year":"2018","journal-title":"Curr. 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