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Vascular defects associated with hereditary hemorrhagic telangiectasia revealed in patient-derived isogenic iPSCs in 3D vessels on chip

Hereditary hemorrhagic telangiectasia (HHT) is a genetic disease characterized by weak blood vessels. HHT1 is caused by mutations in the ENDOGLIN (ENG) gene. Here, we generated induced pluripotent stem cells (hiPSCs) from a patient with rare mosaic HHT1 with tissues containing both mutant (ENG(c.167...

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Detalles Bibliográficos
Autores principales: Orlova, Valeria V., Nahon, Dennis M., Cochrane, Amy, Cao, Xu, Freund, Christian, van den Hil, Francijna, Westermann, Cornelius J.J., Snijder, Repke J., Ploos van Amstel, Johannes Kristian, ten Dijke, Peter, Lebrin, Franck, Mager, Hans-Jurgen, Mummery, Christine L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9287680/
https://www.ncbi.nlm.nih.gov/pubmed/35777360
http://dx.doi.org/10.1016/j.stemcr.2022.05.022