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Old 06-03-2014
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Default Marfan's syndrome

What is the role of TGF-B in causing cystic medial necrosis in aorta ?
I cannot understand this..please explain !
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Old 06-03-2014
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Quote:
Originally Posted by ANUBHAV View Post
What is the role of TGF-B in causing cystic medial necrosis in aorta ?
I cannot understand this..please explain !

hello anubhav.!

Connective tissue is made up of proteins. The protein that plays a role in Marfan syndrome is called fibrillin-1. Marfan syndrome is caused by a defect (or mutation) in the gene that tells the body how to make fibrillin-1. This mutation results in an increase in a protein called transforming growth factor beta, or TGF-β. The increase in TGF-β causes problems in connective tissues throughout the body, which in turn creates the features and medical problems associated with Marfan syndrome and some related disorders.
Marfan syndrome present with a pneumothorax secondary to rupture of emphysematous bullae in the lung.fibrillin-1 functions to limit the activation of TGF-beta, whereas fibrillin-1 deficiency causes excessive amounts of active TGF-beta to be liberated from the matrix. Thus, it seems that just the right extracellular concentration and activity of the correct isoform of TGF-beta at just the right moment is essential to normal lung development.

thus secondary to mutated structural fibrillin, excessive TGF-β at the lungs, heart valves, and aorta weakens the tissues and causes the features of Marfan syndrome.
angiotensin II receptor antagonists (ARBs) also reduce TGF-β, ARBs (such as losartan) have been tested in a small sample of young, severely affected people with Marfan syndrome
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Old 06-03-2014
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Even i am not able to understand it.
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Old 06-03-2014
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Quote:
Originally Posted by drneelema View Post
hello anubhav.!

Connective tissue is made up of proteins. The protein that plays a role in Marfan syndrome is called fibrillin-1. Marfan syndrome is caused by a defect (or mutation) in the gene that tells the body how to make fibrillin-1. This mutation results in an increase in a protein called transforming growth factor beta, or TGF-β. The increase in TGF-β causes problems in connective tissues throughout the body, which in turn creates the features and medical problems associated with Marfan syndrome and some related disorders.
Marfan syndrome present with a pneumothorax secondary to rupture of emphysematous bullae in the lung.fibrillin-1 functions to limit the activation of TGF-beta, whereas fibrillin-1 deficiency causes excessive amounts of active TGF-beta to be liberated from the matrix. Thus, it seems that just the right extracellular concentration and activity of the correct isoform of TGF-beta at just the right moment is essential to normal lung development.

thus secondary to mutated structural fibrillin, excessive TGF-β at the lungs, heart valves, and aorta weakens the tissues and causes the features of Marfan syndrome.
angiotensin II receptor antagonists (ARBs) also reduce TGF-β, ARBs (such as losartan) have been tested in a small sample of young, severely affected people with Marfan syndrome
Sorry , but I want to know how does excess TGF-B cause cystic medial necrosis.
and also if anybody can tell the REASON for BONE OVERGROWTH in marfan's.
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