{"doi":"10.1016/j.celrep.2021.109601","title":"The non-muscle ADF/cofilin-1 controls sarcomeric actin filament integrity and force production in striated muscle laminopathies","abstract":"Cofilins are important for the regulation of the actin cytoskeleton, sarcomere organization, and force production. The role of cofilin-1, the non-muscle-specific isoform, in muscle function remains unclear. Mutations in LMNA encoding A-type lamins, intermediate filament proteins of the nuclear envelope, cause autosomal Emery-Dreifuss muscular dystrophy (EDMD). Here, we report increased cofilin-1 expression in LMNA mutant muscle cells caused by the inability of proteasome degradation, suggesting a protective role by ERK1/2. It is known that phosphorylated ERK1/2 directly binds to and catalyzes phosphorylation of the actin-depolymerizing factor cofilin-1 on Thr25. In vivo ectopic expression of cofilin-1, as well as its phosphorylated form on Thr25, impairs sarcomere structure and force generation. These findings present a mechanism that provides insight into the molecular pathogenesis of muscular dystrophies caused by LMNA mutations.","journal":"Cell Reports","year":2021,"id":190248,"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":14,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9464,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2021-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":753823,"name":"Maria Chatzifrangkeskou","orcid":"0000-0003-3703-491X","position":1,"is_corresponding":false},{"id":754299,"name":"Luca Pinton","orcid":null,"position":2,"is_corresponding":false},{"id":753824,"name":"Hugo Wioland","orcid":"0000-0001-5254-9642","position":3,"is_corresponding":false},{"id":753825,"name":"Thibaut Marais","orcid":"0000-0002-1968-193X","position":4,"is_corresponding":false},{"id":754300,"name":"Mégane Lemaître","orcid":null,"position":5,"is_corresponding":false},{"id":753826,"name":"Caroline Le Dour","orcid":"0000-0001-8647-3252","position":6,"is_corresponding":false},{"id":366840,"name":"Cécile Peccate","orcid":"0000-0002-4125-3616","position":7,"is_corresponding":false},{"id":753827,"name":"Déborah Cardoso","orcid":"0000-0002-6594-3240","position":8,"is_corresponding":false},{"id":753828,"name":"Alain Schmitt","orcid":"0000-0002-3565-8692","position":9,"is_corresponding":false},{"id":601007,"name":"Wei Wu","orcid":"0000-0001-6984-3581","position":10,"is_corresponding":false},{"id":753829,"name":"Maria Grazia Biferi","orcid":"0000-0003-3722-4529","position":11,"is_corresponding":false},{"id":753830,"name":"Naïra Naouar","orcid":"0000-0003-2161-8603","position":12,"is_corresponding":false},{"id":754301,"name":"Coline Macquart","orcid":null,"position":13,"is_corresponding":false},{"id":753831,"name":"Maud Beuvin","orcid":"0000-0002-5908-761X","position":14,"is_corresponding":false},{"id":593666,"name":"V. Decostre","orcid":"0000-0001-8509-9107","position":15,"is_corresponding":false},{"id":563347,"name":"Gisèle Bonne","orcid":"0000-0002-2516-3258","position":16,"is_corresponding":false},{"id":753832,"name":"Guillaume Romet‐Lemonne","orcid":"0000-0002-4938-1065","position":17,"is_corresponding":false},{"id":493357,"name":"Howard J. Worman","orcid":null,"position":18,"is_corresponding":false},{"id":753833,"name":"Francesco Saverio Tedesco","orcid":"0000-0001-5321-7682","position":19,"is_corresponding":false},{"id":682388,"name":"Antoine Jégou","orcid":"0000-0003-0356-3127","position":20,"is_corresponding":false},{"id":316779,"name":"Antoine Muchir","orcid":"0000-0002-4780-9275","position":21,"is_corresponding":false},{"id":753822,"name":"Nicolas Vignier","orcid":"0000-0002-9410-9327","position":0,"is_corresponding":true}],"reference_count":64,"raw_metadata":null,"created_at":"2026-07-18T23:49:22.671571Z","pmid":"34433058","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":[]}