TGFβ-2 haploinsufficiency causes early death in mice with Marfan syndrome

•TGF beta2 heterozygosity synergizes with fibrillin1 hypomorphism to cause early death in mice.•Death is not from dissection and rupture.•Mortality is associated with abnormal aortic valves. To assess the contribution of individual TGF-β isoforms to aortopathy in Marfan syndrome (MFS), we quantified...

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Veröffentlicht in:	Matrix biology 2023-08, Vol.121, p.41-55
Hauptverfasser:	Sachan, Nalani, Phoon, Colin K.L., Zilberberg, Lior, Kugler, Matthias C., Ene, Taylor, Mintz, Shana B., Murtada, Sae-Il, Weiss, Dar, Fishman, Glenn I., Humphrey, Jay D., Rifkin, Daniel B.
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Aorta Aortic regurgitation Aortic valves Biomechanical evaluation Fibrillin-1 - genetics Haploinsufficiency Marfan syndrome Marfan Syndrome - genetics Mice Phenotype TGF-β2 Thoracic aortic aneurysms Transforming Growth Factor beta2 - genetics
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