Abstract
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-ß signalling. It is now known that the disease is caused by altered regulation of TGF-ß. 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.
| Bidragets oversatte titel | [Molecular biological aspects of Marfan syndromes] |
|---|---|
| Originalsprog | Dansk |
| Tidsskrift | Ugeskrift for Laeger |
| Vol/bind | 173 |
| Udgave nummer | 5 |
| Sider (fra-til) | 333-7 |
| Antal sider | 5 |
| ISSN | 0041-5782 |
| Status | Udgivet - 2011 |