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Development of preventive medicine for aortic aneurysm and dissection of Marfan syndrome

update:2020-06-16
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Marfan’s syndrome (MF) is a severe, systemic disorder of connective tissue formation. A variety of MFS therapies have been developed, including surgical therapy for aortic root aneurysm and dissection (AAD) that are life-threatening, traditional medical therapies such as β-adrenergic receptor or angiotensin II receptor blockers for slow aortic growth and to decrease the risk of AAD. However these treatments did not prevent tissue destruction in the AAD of MF. To develop preventive strategy for AAD, it will be necessary to identify molecular mechanisms of microfibril formation and an appropriate fibrillin-1 microfibril associated molecule. Recently, ADAMTSL6β, which is a microfibril-associated extracellular matrix protein contributes the regeneration of microfibril by promoting fibrillin-microfibril assembly. We try to develop ADAMTSL6β as a preventive medicine for AAD of MF by regeneration of fibrillin-1 microfibril assembly.

Researchers

Graduate School of Dentistry, Division of Operative Dentistry, Department of Restorative Dentistry

SAITOU Masahiro , Professor
Doctor of Dentistry

Keywords

Related Information

M.Saito, T.Tsuji, Extracellular matrix administration as a potential therapeutic strategy for periodontal ligament regeneration. Expert Opin Biol Ther, Mar;12(3):299-309, 2012

M. Saito, M. Kurokawa, M. Oda, M. Oshima, K. Tsutsui, K. Kosaka, K. Nakao, M. Ogawa, R. Manabe, N. Suda, G. Ganjargal, Y. Hada, T. Noguchi, T. Teranaka, K. Sekiguchi, T. Yoneda and T. Tsuji. ADAMTSL6Lβ rescues fibrillin-1 microfibril disorder in Marfan syndrome mouse model through the promotion of fibrillin-1 assembly. J Biol Chem. 2011 4;286(44):38602-38613
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