[HTML][HTML] Betaglycan (TGFBR3) up-regulation correlates with increased TGF-β signaling in Marfan patient fibroblasts in vitro
ME Groeneveld, N Bogunovic, RJP Musters… - Cardiovascular …, 2018 - Elsevier
Background Marfan syndrome (MFS), a congenital connective tissue disorder leading to
aortic aneurysm development, is caused by fibrillin-1 (FBN1) gene mutations. Transforming
growth factor beta (TGF-β) might play a role in the pathogenesis. It is still a matter of
discussion if and how TGF-β up-regulates the intracellular downstream pathway, although
TGF-β receptor 3 (TGFBR3 or Betaglycan) is thought to be involved. We aimed to elucidate
the role of TGFBR3 protein in TGF-β signaling in Marfan patients. Methods Dermal …
aortic aneurysm development, is caused by fibrillin-1 (FBN1) gene mutations. Transforming
growth factor beta (TGF-β) might play a role in the pathogenesis. It is still a matter of
discussion if and how TGF-β up-regulates the intracellular downstream pathway, although
TGF-β receptor 3 (TGFBR3 or Betaglycan) is thought to be involved. We aimed to elucidate
the role of TGFBR3 protein in TGF-β signaling in Marfan patients. Methods Dermal …
