{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Zhang RM"],"funding":["Natural Sciences and Engineering Research Council of Canada","China Scholarship Council","Marfan Foundation","Canadian Institutes of Health Research","CIHR"],"pagination":["314"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC11072253"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["79(6)"],"pubmed_abstract":["Thoracic aortic aneurysms (TAA) in Marfan syndrome, caused by fibrillin-1 mutations, are characterized by elevated cytokines and fragmentated elastic laminae in the aortic wall. This study explored whether and how specific fibrillin-1-regulated miRNAs mediate inflammatory cytokine expression and elastic laminae degradation in TAA. miRNA expression profiling at early and late TAA stages using a severe Marfan mouse model (Fbn1<sup>mgR/mgR</sup>) revealed a spectrum of differentially regulated miRNAs. Bioinformatic analyses predicted the involvement of these miRNAs in inflammatory and extracellular matrix-related pathways. We demonstrate that upregulation of pro-inflammatory cytokines and matrix metalloproteinases is a common characteristic of mouse and human TAA tissues. miR-122, the most do"],"journal":["Cellular and molecular life sciences : CMLS"],"pubmed_title":["Fibrillin-1-regulated miR-122 has a critical role in thoracic aortic aneurysm formation."],"pmcid":["PMC11072253"],"funding_grant_id":["PJT-162099","RGPIN-06278"],"pubmed_authors":["Muthu ML","Dinesh NEH","Reinhardt DP","Zhang RM","Komarova S","Tiedemann K","Ramkhelawon B"],"additional_accession":[]},"is_claimable":false,"name":"Fibrillin-1-regulated miR-122 has a critical role in thoracic aortic aneurysm formation.","description":"Thoracic aortic aneurysms (TAA) in Marfan syndrome, caused by fibrillin-1 mutations, are characterized by elevated cytokines and fragmentated elastic laminae in the aortic wall. This study explored whether and how specific fibrillin-1-regulated miRNAs mediate inflammatory cytokine expression and elastic laminae degradation in TAA. miRNA expression profiling at early and late TAA stages using a severe Marfan mouse model (Fbn1<sup>mgR/mgR</sup>) revealed a spectrum of differentially regulated miRNAs. Bioinformatic analyses predicted the involvement of these miRNAs in inflammatory and extracellular matrix-related pathways. We demonstrate that upregulation of pro-inflammatory cytokines and matrix metalloproteinases is a common characteristic of mouse and human TAA tissues. miR-122, the most do","dates":{"release":"2022-01-01T00:00:00Z","publication":"2022 May","modification":"2026-06-01T20:26:06.63Z","creation":"2026-05-22T03:07:44.052Z"},"accession":"S-EPMC11072253","cross_references":{"pubmed":["35606547"],"doi":["10.1007/s00018-022-04337-8"]}}