TGF-beta-dependent mechanisms of patho genesis of Marfan syndrome and related disorders
https://doi.org/10.18705/1607-419X-2009-15-2-223-226
Abstract
Recent research on the molecular physiology of fibrillin and the pathophysiology of Marfan syndrome and related connective tissue disorders has changed our understanding of this pathology by demonstrating changes in growth factor signalling and in matrix-cell interactions. Marfan syndrome is an autosomal dominant disorder of connective tissue caused by mutations in fibrillin-1. Fibrillin-1 contributes to the regulated activation of the cytokine TGF-ß, and enhanced signaling is a consequence of fibrillin-1 deficiency. Thereby, increased TGF-ß signaling may contribute to the multisystem pathogenesis of Marfan syndrome, including the development of myxomatous changes of the atrioventricular valve, aortic aneurysm and dissection, joint hypermobility syndrome. These data suggest that anti-TGF-β therapeutic strategy for patients with Marfan syndrome can be useful in prevention of the major life-threatening manifestation of this disorder.
About the Author
A. S. Rudoy
Public institution «432 awards of the Red Star the main clinical medical military center of the Armed Forces of the Republic of Belarus»
Russian Federation
Russian Federation
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Review
For citations:
Rudoy A.S. TGF-beta-dependent mechanisms of patho genesis of Marfan syndrome and related disorders. "Arterial’naya Gipertenziya" ("Arterial Hypertension"). 2009;15(2):223-226. (In Russ.) https://doi.org/10.18705/1607-419X-2009-15-2-223-226
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