Submitted by Administrator on Fri, 09/06/2017 - 12:48
The BHF has awarded Dr Sanjay Sinha and Dr Paul Bertone a £1,270,000 programme grant over five years to identify new therapeutic strategies for genetically-triggered thoracic aortic aneurysm syndromes (Marfan, Loeys-Dietz, Ehlers-Danlos and ACTA2).
Although rupture or dissection of these aneurysms can have catastrophic consequences, there are no specific medical treatments available - likely due to an incomplete understanding of the molecular mechanisms leading to aortic wall weakness.
Dr Sinha's group has pioneered a human induced pluripotent stem cell (hiPSC)-based in vitro model of Marfan's that recapitulates the aneurysm phenotype and has identified a complex network of signalling defects including p38 mitogen-activated protein kinase as a novel regulator of vascular smooth muscle cell (SMC) death.
Using this unique hiPSC-based model of Marfan and other aortic aneurysm syndromes together with murine models, Dr Sinha's and Dr Bertone's groups will determine common disease-causing pathways and identify potential new treatments.
