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. Author manuscript; available in PMC: 2013 Jan 4.
Published in final edited form as: FEBS Lett. 2012 May 26;586(14):2003–2015. doi: 10.1016/j.febslet.2012.05.027

Figure 1.

Figure 1

Integrated signaling cascades in Marfan syndrome and related diseases. Canonical (green) and noncanonical (blue) TGFβ signaling cascades, as well as intracellular proteins not currently implicated in downstream TGFβ signaling (red), are illustrated according. Disorders caused by a mutated gene product are shown in brackets next to the protein. Drugs shown in green have been tested in Marfan mice and/or patients. Drugs shown in red are untested but may have hypothetical benefit, based on our current understanding of disease pathogenesis. MFS: Marfan syndrome; SSS: Stiff skin syndrome; WMS: Weill-Marchesani syndrome; ELS: Ectopia lentis syndrome; GD: Geophysic dysplasia; AC: Acromelic dysplasia; HHT T1: Hereditary hemorrhagic telangiectasia Type 1; HHT T2: Hereditary hemorrhagic telangiectasia Type 2; ATS: Arterial tortuosity syndrome; PVNH: Periventricular nodular heterotopia; LDS: Loeys Dietz syndrome; iFTAA: Isolated familial thoracic aortic aneurysm; FSS: Ferguson Smith syndrome. NAb: neutralizing antibody; ARB: Angiotensin II Type 1 receptor blocker (e.g. losartan); ACEi: Angiotensin-converting enzyme inhibitor (e.g. enalapril).