{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Wu T"],"funding":["NHLBI NIH HHS","National Heart, Lung, and Blood Institute","NINDS NIH HHS"],"pagination":["e1010630"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC9907827"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["19(1)"],"pubmed_abstract":["FLNC, encoding filamin C, is one of the most mutated genes in dilated and hypertrophic cardiomyopathy. However, the precise role of filamin C in mammalian heart remains unclear. In this study, we demonstrated Flnc global (FlncgKO) and cardiomyocyte-specific knockout (FlnccKO) mice died in utero from severely ruptured ventricular myocardium, indicating filamin C is required to maintain the structural integrity of myocardium in the mammalian heart. Contrary to the common belief that filamin C acts as an integrin inactivator, we observed attenuated activation of β1 integrin specifically in the myocardium of FlncgKO mice. Although deleting β1 integrin from cardiomyocytes did not recapitulate the heart rupture phenotype in Flnc knockout mice, deleting both β1 integrin and filamin C from cardiom"],"journal":["PLoS genetics"],"pubmed_title":["Filamin C is Essential for mammalian myocardial integrity."],"pmcid":["PMC9907827"],"funding_grant_id":["P30 NS047101","R01 HL157119","R01 HL153032","R01 HL144872","R01 HL155826","R01HL144872","T32 HL007444","R01 HL158981","R01 HL146759"],"pubmed_authors":["Chen J","Liang Z","Wu T","Zhang L","Evans SM","Zhou X","Xu Y"],"additional_accession":[]},"is_claimable":false,"name":"Filamin C is Essential for mammalian myocardial integrity.","description":"FLNC, encoding filamin C, is one of the most mutated genes in dilated and hypertrophic cardiomyopathy. However, the precise role of filamin C in mammalian heart remains unclear. In this study, we demonstrated Flnc global (FlncgKO) and cardiomyocyte-specific knockout (FlnccKO) mice died in utero from severely ruptured ventricular myocardium, indicating filamin C is required to maintain the structural integrity of myocardium in the mammalian heart. Contrary to the common belief that filamin C acts as an integrin inactivator, we observed attenuated activation of β1 integrin specifically in the myocardium of FlncgKO mice. Although deleting β1 integrin from cardiomyocytes did not recapitulate the heart rupture phenotype in Flnc knockout mice, deleting both β1 integrin and filamin C from cardiom","dates":{"release":"2023-01-01T00:00:00Z","publication":"2023 Jan","modification":"2026-05-28T12:46:47.038Z","creation":"2025-04-05T19:41:26.096Z"},"accession":"S-EPMC9907827","cross_references":{"pubmed":["36706168"],"doi":["10.1371/journal.pgen.1010630"]}}