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Klin Lab Diagn ; 61(2): 103-6, 2016 Feb.
Article in Russian | MEDLINE | ID: mdl-27455564

ABSTRACT

The recent studies of molecular physiology of fibrillin and pathophysiology of inherent disorders of structure and function of connective tissue such as dissection and aneurysm of aorta, myxomatously altered cusps and prolapses of mitral valve, syndrome of hyper-mobility of joints, demonstrated that important role in development of these malformations play alterations of transfer of signals by growth factors and matrix cellular interaction. These conditions under manifesting Marfan's syndrome can be a consequence of anomalies of fibrillin-1 which deficiency unbrakes process of activation of transforming growth factor-ß (TGFß). The involvement of TGFß in pathogenesis of Marfan's syndrome permits consider antagonists of angiotensin-transforming enzymes as potential pharmaceuticals in therapy of this disease. The article presents analysis of publications' data related to this problem.


Subject(s)
Aortic Aneurysm/immunology , Aortic Dissection/immunology , Joint Instability/immunology , Marfan Syndrome/immunology , Mitral Valve Prolapse/immunology , Transforming Growth Factor beta/immunology , Aortic Dissection/drug therapy , Aortic Dissection/genetics , Aortic Dissection/pathology , Angiotensin-Converting Enzyme Inhibitors/therapeutic use , Aortic Aneurysm/drug therapy , Aortic Aneurysm/genetics , Aortic Aneurysm/pathology , Connective Tissue/drug effects , Connective Tissue/immunology , Connective Tissue/pathology , Fibrillin-1 , Fibrillins , Gene Expression Regulation , Humans , Joint Instability/drug therapy , Joint Instability/genetics , Joint Instability/pathology , Marfan Syndrome/drug therapy , Marfan Syndrome/genetics , Marfan Syndrome/pathology , Microfilament Proteins/genetics , Microfilament Proteins/immunology , Mitral Valve Prolapse/drug therapy , Mitral Valve Prolapse/genetics , Mitral Valve Prolapse/pathology , Peptidyl-Dipeptidase A/genetics , Peptidyl-Dipeptidase A/immunology , Signal Transduction , Transforming Growth Factor beta/genetics
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