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Marfan Syndrome and Related Disorders: from Molecules to Medicines

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Air date: Wednesday, January 14, 2009, 3:00:00 PM
Time displayed is Eastern Time, Washington DC Local
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Category: Wednesday Afternoon Lectures
Runtime: 01:01:38
Description: Our goal is to understand the genetic factors that predispose to aortic aneurysm, a condition accounting for 1–2 percent of deaths in industrialized countries. We have shown that the growth factor molecule TGF-beta drives many of the manifestations of Marfan syndrome, including aortic aneurysm, developmental emphysema, myxomatous valve changes and skeletal myopathy. Most importantly, these findings can be attenuated or prevented in validated animal models through TGF-beta antagonism in vivo.

The identical mechanism and therapeutic response is relevant to other more common presentations of these important disease phenotypes. These data are illustrative of the promise that disease gene identification and elucidation of pathogenesis for rare Mendelian disorders will lead to the development of novel therapeutic strategies with broad application.

The NIH Director's Wednesday Afternoon Lecture Series includes weekly scientific talks by some of the top researchers in the biomedical sciences worldwide.
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NLM Title: Marfan syndrome and related disorders : from molecules to medicines [electronic resource] / Harry C. Dietz III.
Series: Astute clinican lecture ; 11th
Author: Dietz, Harry C.
National Institutes of Health (U.S.)
Publisher:
Other Title(s): Astute clinican lecture ; 11th
Abstract: (CIT): Our goal is to understand the genetic factors that predispose to aortic aneurysm, a condition accounting for 1-2 percent of deaths in industrialized countries. We have shown that the growth factor molecule TGF-beta drives many of the manifestations of Marfan syndrome, including aortic aneurysm, developmental emphysema, myxomatous valve changes and skeletal myopathy. Most importantly, these findings can be attenuated or prevented in validated animal models through TGF-beta antagonism in vivo. The identical mechanism and therapeutic response is relevant to other more common presentations of these important disease phenotypes. These data are illustrative of the promise that disease gene identification and elucidation of pathogenesis for rare Mendelian disorders will lead to the development of novel therapeutic strategies with broad application.
Subjects: Angiotensin II Type 1 Receptor Blockers
Aortic Aneurysm--genetics
Marfan Syndrome
Microfilament Proteins
Transforming Growth Factor beta
Publication Types: Lectures
Webcasts
Download: To download this event, select one of the available bitrates:
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NLM Classification: WD 375
NLM ID: 101496160
CIT Live ID: 7027
Permanent link: http://videocast.nih.gov/launch.asp?14857

 

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