biostudies-literatureBallarino MFondazione Italiana di Ricerca per la Sclerosi Laterale AmiotroficaHuman Frontier Science ProgramTelethonEuropean Research CouncilMedical Research CouncilSapienza Università di RomaAFM-Téléthone99697https://www.ebi.ac.uk/biostudies/studies/S-EPMC6138438biostudies-literatureUnknown37(18)Myogenesis is a highly regulated process that involves the conversion of progenitor cells into multinucleated myofibers. Besides proteins and miRNAs, long noncoding RNAs (lncRNAs) have been shown to participate in myogenic regulatory circuitries. Here, we characterize a murine chromatin-associated muscle-specific lncRNA, Charme, which contributes to the robustness of the myogenic program in vitro and in vivo In myocytes, Charme depletion triggers the disassembly of a specific chromosomal domain and the downregulation of myogenic genes contained therein. Notably, several Charme-sensitive genes are associated with human cardiomyopathies and Charme depletion in mice results in a peculiar cardiac remodeling phenotype with changes in size, structure, and shape of the heart. Moreover, the existence of an orthologous transcript in human, regulating the same subset of target genes, suggests an important and evolutionarily conserved function for Charme Altogether, these data describe a new example of a chromatin-associated lncRNA regulating the robustness of skeletal and cardiac myogenesis.The EMBO journalDeficiency in the nuclear long noncoding RNA Charme causes myogenic defects and heart remodeling in mice.PMC6138438GGP14066GGP16213AdG 340172 MUNCODDGTB12001F34017217835GA310206ARCIMR/K017047/1RGP0009/2014RM11715C7C8176C1Ballarino MTita RCarrieri CDesideri FNicoletti CColantoni ASantini TBozzoni ICipriano AMusaro ACarroll DOMorlando MfalseDeficiency in the nuclear long noncoding RNA Charme causes myogenic defects and heart remodeling in mice.Myogenesis is a highly regulated process that involves the conversion of progenitor cells into multinucleated myofibers. Besides proteins and miRNAs, long noncoding RNAs (lncRNAs) have been shown to participate in myogenic regulatory circuitries. Here, we characterize a murine chromatin-associated muscle-specific lncRNA, Charme, which contributes to the robustness of the myogenic program in vitro and in vivo In myocytes, Charme depletion triggers the disassembly of a specific chromosomal domain and the downregulation of myogenic genes contained therein. Notably, several Charme-sensitive genes are associated with human cardiomyopathies and Charme depletion in mice results in a peculiar cardiac remodeling phenotype with changes in size, structure, and shape of the heart. Moreover, the existence of an orthologous transcript in human, regulating the same subset of target genes, suggests an important and evolutionarily conserved function for Charme Altogether, these data describe a new example of a chromatin-associated lncRNA regulating the robustness of skeletal and cardiac myogenesis.2018-01-01T00:00:00Z2018 Sep2026-04-30T03:12:42.294Z2019-03-26T23:56:08ZS-EPMC61384383017757210.15252/embj.201899697