ArticleArteriosclerosis, thrombosis, and vascular biology2023
IL11 (Interleukin-11) Causes Emphysematous Lung Disease in a Mouse Model of Marfan Syndrome.
Article in Arteriosclerosis, thrombosis, and vascular biology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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Who cites it
7 citing papers in PubMed, 13 citations in OpenAlex.
- The hypoxic tight-skin mouse model of Group 3 pulmonary hypertension.Scientific reports · 2026Article
- Identification and functional validation of a novel FBN1 variant in a Marfan syndrome family using a zebrafish model.BMC genomics · 2025Article
- Emerging roles for interleukin-11 in the inflammatory response of intracerebral hemorrhage.Frontiers in neurology · 2025Review
- The Impact of Liver Failure on the Immune System.International journal of molecular sciences · 2024Review
- Understanding interleukin 11 as a disease gene and therapeutic target.The Biochemical journal · 2023Review
- Gingival Fibroblasts Are Sensitive to Oral Cell Lysates Indicated by Their IL11 Expression.Bioengineering (Basel, Switzerland) · 2023Article
- Elucidating the dynamic immune responses within the ocular mucosa of rainbow trout (Frontiers in immunology · 2023Article
Corrections and comments
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Authors and funding
9 authors at 2 institutions in 3 countries.
Funding
Abstract
backgroundMarfan Syndrome (MFS) is an inherited connective tissue disorder caused by mutations in the FBN1 (fibrillin-1) gene. Lung abnormalities are common in MFS, but their pathogenesis is poorly understood. IL11 (interleukin-11) causes aortic disease in a mouse model of MFS and was studied here in the lung.
methodsWe examined histological and molecular phenotypes in the lungs of
resultsmMFS lungs showed progressive loss and enlargement of distal airspaces associated with increased proinflammatory and profibrotic gene expression as well as matrix metalloproteinases 2, 9, and 12. IL11 was increased in mMFS lungs and localized to smooth muscle and endothelial cells in young mMFS mice in the
conclusionsIL11 causes lung disease in mMFS. This reveals a shared IL11-driven disease mechanism in lung and aorta in MFS and suggests inhibition of IL11 signaling as a holistic approach for treating multiorgan morbidity in MFS.
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Registered trials
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