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It is caused by the excessive expansion of noncoding CTG repeats, which when transcribed affects the functions of RNA\u2010binding factors with adverse effects on alternative splicing, processing, and stability of a large set of muscular and cardiac transcripts. Among these effects, inefficient processing and down\u2010regulation of muscle\u2010 and heart\u2010specific miRNA,\n                    <jats:italic>miR\u20101<\/jats:italic>\n                    , have been reported in DM1 patients, but the impact of reduced\n                    <jats:italic>miR\u20101<\/jats:italic>\n                    on DM1 pathogenesis has been unknown. Here, we use\n                    <jats:italic>Drosophila<\/jats:italic>\n                    DM1 models to explore the role of\n                    <jats:italic>miR\u20101<\/jats:italic>\n                    in cardiac dysfunction in DM1. We show that\n                    <jats:italic>miR\u20101<\/jats:italic>\n                    down\u2010regulation in the heart leads to dilated cardiomyopathy (DCM), a DM1\u2010associated phenotype. We combined\n                    <jats:italic>in silico<\/jats:italic>\n                    screening for\n                    <jats:italic>miR\u20101<\/jats:italic>\n                    targets with transcriptional profiling of DM1 cardiac cells to identify\n                    <jats:italic>miR\u20101<\/jats:italic>\n                    target genes with potential roles in DCM. We identify Multiplexin (Mp) as a new cardiac\n                    <jats:italic>miR\u20101<\/jats:italic>\n                    target involved in DM1.\n                    <jats:italic>Mp<\/jats:italic>\n                    encodes a collagen protein involved in cardiac tube formation in\n                    <jats:italic>Drosophila<\/jats:italic>\n                    . Mp and its human ortholog Col15A1 are both highly enriched in cardiac cells of DCM\u2010developing DM1 flies and in heart samples from DM1 patients with DCM, respectively. When overexpressed in the heart, Mp induces DCM, whereas its attenuation rescues the DCM phenotype of aged DM1 flies. Reduced levels of\n                    <jats:italic>miR\u20101<\/jats:italic>\n                    and consecutive up\u2010regulation of its target Mp\/Col15A1 might be critical in DM1\u2010associated DCM.\n                  <\/jats:p>","DOI":"10.15252\/embr.202256616","type":"journal-article","created":{"date-parts":[[2023,2,28]],"date-time":"2023-02-28T08:17:19Z","timestamp":1677572239000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Deregulations of miR\u20101 and its target Multiplexin promote dilated cardiomyopathy associated with myotonic dystrophy type 1"],"prefix":"10.1038","volume":"24","author":[{"given":"Anissa","family":"Souidi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masayuki","family":"Nakamori","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6174-2719","authenticated-orcid":false,"given":"Monika","family":"Zmojdzian","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9277-6089","authenticated-orcid":false,"given":"Teresa","family":"Jagla","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoan","family":"Renaud","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4965-8818","authenticated-orcid":false,"given":"Krzysztof","family":"Jagla","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2023,2,28]]},"reference":[{"key":"BFEMBR202256616_CR1","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.51114","volume":"8","author":"E Auxerre\u2010Planti\u00e9","year":"2019","unstructured":"Auxerre\u2010Planti\u00e9 E, Nakamori M, Renaud Y, Huguet A, Choquet C, Dondi C, Miquerol L, Takahashi MP, Gourdon G, Junion G et al (2019) Straightjacket\/\u03b12\u03b43 deregulation is associated with cardiac conduction defects in myotonic dystrophy type 1. 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