Article

A phenotypic screen of Marfan syndrome iPSC-derived vascular smooth muscle cells uncovers GSK3β as a new target

2022-04-11

Abstract excerpt

Marfan syndrome (MFS) is a rare connective tissue disorder caused by mutations in FBN1 . Patients with MFS notably suffer from aortic aneurysm and dissection. Despite considerable effort, animal models have proven to be poorly predictive for therapeutic intervention in human aortic disease. Using a “humanised” model system may be more appropriate in identifying new therapeutic targets. Patient-derived induced plu...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: 6b69bf70-6d47-54f8-bbb6-f45e4254482b
DOI: 10.1101/2022.04.11.487841

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

A phenotypic screen of Marfan syndrome iPSC-derived vascular smooth muscle cells uncovers GSK3β as a new targetDOI 10.1101/2022.04.11.487841Read

A phenotypic screen of Marfan syndrome iPSC-derived vascular smooth muscle cells uncovers GSK3β as a new targetSemantic proximity Read

An iPSC-derived vascular model of Marfan syndrome identifies key mediators of smooth muscle cell deathSemantic proximity Read

Development of novel therapies for Marfan syndrome using a human iPSC-disease modelSemantic proximity Read

Quantitative proteomics reveal lineage-specific protein profiles in iPSC-derived Marfan syndrome smooth muscle cellsSemantic proximity Read

Clinical, diagnostic, and therapeutic aspects of the Marfan syndrome.Semantic proximity Read

Marfan Syndrome and Related Disorders: 25 Years of Gene Discovery.Semantic proximity Read

A Marfan syndrome human induced pluripotent stem cell line with a heterozygous FBN1 c.4082G>A mutation, ISMMSi002-B, for disease modeling.Semantic proximity Read

Endothelial Cell Senescence in Marfan Syndrome: Pathogenesis and Therapeutic Potential of TGF-β Pathway Inhibition.Semantic proximity Read

Marfan syndrome: from gene to therapy.Semantic proximity Read

Generation of Marfan syndrome-specific induced pluripotent stem cells harboring FBN1 mutations.Semantic proximity Read

Marfan Patient iPSC-Derived Endothelial Cells Carrying FBN1 Variants Reveal Endothelial DysfunctionSemantic proximity Read

Single-Cell Transcriptomic Profiling of Vascular Smooth Muscle Cell Phenotype Modulation in Marfan Syndrome Aortic Aneurysm.Semantic proximity Read