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- W2172085460 abstract "Marfan syndrome (MFS), a relatively common autosomal dominant hereditary disorder of connective tissue with prominent manifestations in the skeletal, ocular, and cardiovascular systems, is caused by mutations in the gene for fibrillin-1 (FBN1). The leading cause of premature death in untreated individuals with MFS is acute aortic dissection, which often follows a period of progressive dilatation of the ascending aorta. Recent research on the molecular physiology of fibrillin and the pathophysiology of MFS and related disorders has changed our understanding of this disorder by demonstrating changes in growth factor signalling and in matrix-cell interactions. The purpose of this review is to provide a comprehensive overview of recent advances in the molecular biology of fibrillin and fibrillin-rich microfibrils. Mutations in FBN1 and other genes found in MFS and related disorders will be discussed, and novel concepts concerning the complex and multiple mechanisms of the pathogenesis of MFS will be explained." @default.
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- W2172085460 date "2006-03-29" @default.
- W2172085460 modified "2023-10-10" @default.
- W2172085460 title "The molecular genetics of Marfan syndrome and related disorders" @default.
- W2172085460 cites W1033793070 @default.
- W2172085460 cites W119322828 @default.
- W2172085460 cites W1503363172 @default.
- W2172085460 cites W1507091468 @default.
- W2172085460 cites W1513040830 @default.
- W2172085460 cites W1522309752 @default.
- W2172085460 cites W1530181168 @default.
- W2172085460 cites W1541817349 @default.
- W2172085460 cites W1553005491 @default.
- W2172085460 cites W1555580499 @default.
- W2172085460 cites W1570825587 @default.
- W2172085460 cites W1572528271 @default.
- W2172085460 cites W1572983895 @default.
- W2172085460 cites W1595568818 @default.
- W2172085460 cites W166105045 @default.
- W2172085460 cites W1662680583 @default.
- W2172085460 cites W1833362241 @default.
- W2172085460 cites W1904364980 @default.
- W2172085460 cites W1934555047 @default.
- W2172085460 cites W1962847913 @default.
- W2172085460 cites W1963770529 @default.
- W2172085460 cites W1964724190 @default.
- W2172085460 cites W1964803698 @default.
- W2172085460 cites W1964845795 @default.
- W2172085460 cites W1965361623 @default.
- W2172085460 cites W1965508013 @default.
- W2172085460 cites W1966152952 @default.
- W2172085460 cites W1967286679 @default.
- W2172085460 cites W1967510639 @default.
- W2172085460 cites W1969660279 @default.
- W2172085460 cites W1972843274 @default.
- W2172085460 cites W1976491232 @default.
- W2172085460 cites W1977959731 @default.
- W2172085460 cites W1978719997 @default.
- W2172085460 cites W1980717155 @default.
- W2172085460 cites W1982279766 @default.
- W2172085460 cites W1983313606 @default.
- W2172085460 cites W1984175120 @default.
- W2172085460 cites W1984350356 @default.
- W2172085460 cites W1984451564 @default.
- W2172085460 cites W1984889153 @default.
- W2172085460 cites W1984953746 @default.
- W2172085460 cites W1985387642 @default.
- W2172085460 cites W1985498506 @default.
- W2172085460 cites W1986897852 @default.
- W2172085460 cites W1987521883 @default.
- W2172085460 cites W1988456947 @default.
- W2172085460 cites W1989948980 @default.
- W2172085460 cites W1991309781 @default.
- W2172085460 cites W1991432766 @default.
- W2172085460 cites W1991609451 @default.
- W2172085460 cites W1994876976 @default.
- W2172085460 cites W1995060796 @default.
- W2172085460 cites W1996020433 @default.
- W2172085460 cites W1997216820 @default.
- W2172085460 cites W1999271571 @default.
- W2172085460 cites W1999932869 @default.
- W2172085460 cites W2000003698 @default.
- W2172085460 cites W2000308032 @default.
- W2172085460 cites W2002821131 @default.
- W2172085460 cites W2002886248 @default.
- W2172085460 cites W2005270333 @default.
- W2172085460 cites W2005776119 @default.
- W2172085460 cites W2008663375 @default.
- W2172085460 cites W2009278609 @default.
- W2172085460 cites W2011196754 @default.
- W2172085460 cites W2013100626 @default.
- W2172085460 cites W2013197169 @default.
- W2172085460 cites W2014027189 @default.
- W2172085460 cites W2014876490 @default.
- W2172085460 cites W2014957559 @default.
- W2172085460 cites W2015185847 @default.
- W2172085460 cites W2017009317 @default.
- W2172085460 cites W2017395439 @default.