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SUMMARY:Balancing BMP and TGFβ signaling to maintain cardiac homeostasis 
 and reduce fibrosis: where PAH and HHT meet - Prof. Marie-Jose Goumans Lei
 den University Medical Center in the Netherlands
DTSTART:20240222T130000Z
DTEND:20240222T140000Z
UID:TALK212131@talks.cam.ac.uk
CONTACT:Denise Hatherly
DESCRIPTION:It has become clear that signaling by bone morphogenetic prote
 ins (BMPs)\, which have a long history of studies in bone and early heart 
 development\, are also essential for regulating vascular function. Indeed\
 , loss-of-function mutations that cause deregulated BMP signaling are link
 ed to two distinct human vascular syndromes\, hereditary hemorrhagic telan
 giectasia (HHT) and pulmonary arterial hypertension (PAH). PAH involves ob
 structive remodeling of pulmonary arteries progressing to right heart fail
 ure\, while HHT results in mucocutaneous telangiectasias and visceral arte
 riovenous malformations (AVMs) in various organs\, such as the liver\, lun
 gs\, gastrointestinal tract\, or brain.\n\nAlthough HPAH and HHT exhibit d
 ifferent clinical manifestations\, they share genetic mutations in BMP/TGF
 β signaling pathway-related genes. The factors determining whether these 
 mutations lead to HPAH\, HHT\, or both remain unknown\, as do the cellular
  mechanisms driving each syndrome. In this lecture\, the pivotal role of B
 MPs in the cardiovascular system will be discussed obtained using cells in
  cell culture and mouse models\, as well as the development of an ex vivo 
 heart culture system to model and manipulate TGFβ/BMP signaling during ca
 rdiac fibrosis.
LOCATION: Heart and Lung Institute\, Cambridge Biomedical Campus
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