Circulating Transforming Growth Factor Beta (TGF-β) in Individuals With Marfan Syndrome
Circulating Transforming Growth Factor Beta (TGF-β) in Individuals With Marfan Syndrome
Transforming Growth Factor Beta (TGF-β) is a protein that controls proliferation, cellular
 differentiation, and other functions in most cells. TGF-β levels play a major role in the
 pathogenesis of Marfan syndrome, a disease characterized by disproportionate height, long
 extremities, lens dislocation in the eyes and heart complications such as mitral valve
 prolapse and aortic enlargement increasing the likelihood of aortic dissection. While the
 underlying defect in Marfan syndrome is faulty synthesis of the glycoprotein fibrillin I,
 normally an important component of elastic fibers it has been shown that the Marfan syndrome
 phenotype can be relieved by addition of a TGF-β antagonist in affected mice.
Transforming Growth Factor Beta (TGF-β) is a protein that controls proliferation, cellular
 differentiation, and other functions in most cells. TGF-β levels play a major role in the
 pathogenesis of Marfan syndrome, a disease characterized by disproportionate height, long
 extremities, lens dislocation in the eyes and heart complications such as mitral valve
 prolapse and aortic enlargement increasing the likelihood of aortic dissection. While the
 underlying defect in Marfan syndrome is faulty synthesis of the glycoprotein fibrillin I,
 normally an important component of elastic fibers it has been shown that the Marfan syndrome
 phenotype can be relieved by addition of a TGF-β antagonist in affected mice. This suggest
 that while the symptoms of Marfan syndrome may seem consistent with a connective tissue
 disorder, the mechanism is more likely related to reduced sequestration of TGF-β by
 fibrillin.
Inclusion Criteria:

 -  Individual with Marfan syndrome consented in to the Main Atenolol Vs. Losartan NIH
 study.

 Exclusion Criteria:

 -  Subjects in the main PHN Marfan trial who have not achieved the maintenance drug
 dosing or who have stopped taking study drug.