Robert Wiener

Sep 12, 2024 | nature.com | Sizhao Lu |Yang Xu |Shouneng Peng |Adrien Georges |Aya Nomura-Kitabayashi |Robert Wiener | +5 more

AbstractFibromuscular dysplasia (FMD) is a poorly understood disease affecting 3–5% of adult females. The pathobiology of FMD involves arterial lesions of stenosis, dissection, tortuosity, dilation and aneurysm, which can lead to hypertension, stroke, myocardial infarction and even death. Currently, there are no animal models for FMD and few insights as to its pathobiology.

Oct 4, 2023 | biorxiv.org | Robert Wiener |Kevin Costa

AbstractMarfan Syndrome (MFS), a connective tissue disorder caused by a mutation in the fibrillin-1 gene, occurs in approximately 1 in 5,000 people worldwide. As an important constituent of the extracellular matrix, mutated fibrillin-1 in Marfan Syndrome leads to aortic medial degeneration, aneurysm, and dissection. TGFβ in the matrix, which is controlled by fibrillin-1, is known to cause pathological effects in smooth muscle cells (SMCs) within the aortic wall during MFS.