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Marfan syndrome and marfan-like syndromes.

15:37 EDT 23rd July 2014 | BioPortfolio

Summary of "Marfan syndrome and marfan-like syndromes."

Marfan syndrome (MFS) is a hereditary connective tissue disorder. Studies of MFS have established the critical contribution of fibrillin-1 deficiency to disease progression through altered cell-matrix interactions and dysregulated TGF-β signalling. It is now known that the disease is caused by altered regulation of TGF-β. As a result, the definition of MFS- and MFS-related diseases as the prototypical structural disorder of the connective tissue has changed to that of a developmental abnormality with broad and complex effects on the morphogenesis and tissue remodelling.

Affiliation

Kildegårdsvej 16 B, 2. tv., 2900 Hellerup. t.z.belsing@dadlnet.dk.

Journal Details

This article was published in the following journal.

Name: Ugeskrift for laeger
ISSN: 1603-6824
Pages: 333-337

Links

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Medical and Biotech [MESH] Definitions

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