{"doi":"10.17615/j5fn-wx84","title":"RNA steady-state defects in myotonic dystrophy are linked to nuclear exclusion of SHARP","abstract":"We describe a new mechanism by which CTG tract expansion affects myotonic dystrophy (DM1). Changes to the levels of a panel of RNAs involved in muscle development and function that are downregulated in DM1 are due to aberrant localization of the transcription factor SHARP (SMART/HDAC1-associated repressor protein). Mislocalization of SHARP in DM1 is consistent with increased CRM1-mediated export of SHARP to the cytoplasm. A direct link between CTG repeat expression and SHARP mislocalization is demonstrated as expression of expanded CTG repeats in normal cells recapitulates cytoplasmic SHARP localization. These results demonstrate a role for the inactivation of SHARP transcription in DM1 biology.","journal":"UNC Libraries","year":2020,"id":138089,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":0,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.945,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2020-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":517772,"name":"Paul Marjoram","orcid":"0000-0003-0824-7449","position":1,"is_corresponding":false},{"id":599558,"name":"R Mohammadzadeh","orcid":null,"position":2,"is_corresponding":false},{"id":598804,"name":"Sita Reddy","orcid":"0000-0003-0239-6734","position":3,"is_corresponding":false},{"id":312904,"name":"Warunee Dansithong","orcid":null,"position":4,"is_corresponding":false},{"id":599559,"name":"Sonali P. Jog","orcid":null,"position":5,"is_corresponding":false},{"id":599560,"name":"Yukwah Kwok","orcid":null,"position":6,"is_corresponding":false},{"id":265819,"name":"Rebecca C. Fry","orcid":"0000-0003-0899-9018","position":7,"is_corresponding":false},{"id":599561,"name":"Stephanie Tring","orcid":null,"position":8,"is_corresponding":false},{"id":485377,"name":"Sharan Paul","orcid":"0000-0002-9845-3125","position":9,"is_corresponding":false},{"id":432453,"name":"Lucio Comai","orcid":"0000-0003-3109-1841","position":0,"is_corresponding":true}],"reference_count":0,"raw_metadata":{"citation_network_status":"fetched"},"created_at":"2026-07-18T23:16:59.449905Z","pmid":null,"pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}