Badly engineered fibrillin lessons from molecular studies of marfan syndrome.
Trends Cardiovasc Med
; 7(8): 282-8, 1997 Nov.
Article
de En
| MEDLINE
| ID: mdl-21235897
ABSTRACT
Marfan syndrome (MFS) is one of the most common inherited connective tissue disorders that severely affects the cardiovascular system. Mutations in the gene encoding fibrillin-1 (FBN1) have been shown to cause MFS as well as dominant ectopia lentis and neonatal Marfan syndrome. Fibrillin-1 is the major component of elastic fiber microfibrils in the extracellular matrix of connective tissue. Recent molecular studies have brought some light into understanding the pathogenesis of MFS, but the diagnostic problems still prevail, and targeted therapy of MFS must await better dissection of the role of fibrillin-1 in tissue phenotype using different experimental systems. (Trends Cardiovasc Med 1997;7282-288). © 1997, Elsevier Science Inc.
Texte intégral:
1
Collection:
01-internacional
Base de données:
MEDLINE
Langue:
En
Journal:
Trends Cardiovasc Med
Sujet du journal:
ANGIOLOGIA
/
CARDIOLOGIA
Année:
1997
Type de document:
Article
Pays d'affiliation:
Finlande