title: [THE ROLE OF TRANSFORMING GROWTH FACTOR-B IN IMMUNOPATHOGENESIS OF DISEASES OF CONNECTIVE TISSUE].
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-β (TGFβ). The involvement of TGFβ 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.
authors.forename: A S
authors.initials: AS
authors.lastname: Rudoi
authors.forename: A V
authors.initials: AV
authors.lastname: Moskalev
authors.forename: V B
authors.initials: VB
authors.lastname: Sboitchakov
mesh_terms: D000784:Aortic Dissection / Q000188:drug therapy / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D001014:Aortic Aneurysm / Q000188:drug therapy / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D003238:Connective Tissue / Q000187:drug effects / Q000276:immunology / Q000473:pathology; D000071838:Fibrillin-1; D000071837:Fibrillins; D005786:Gene Expression Regulation; D006801:Humans; D007593:Joint Instability / Q000188:drug therapy / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D008382:Marfan Syndrome / Q000188:drug therapy / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D008840:Microfilament Proteins / Q000235:genetics / Q000276:immunology; D008945:Mitral Valve Prolapse / Q000188:drug therapy / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D007703:Peptidyl-Dipeptidase A / Q000235:genetics / Q000276:immunology; D015398:Signal Transduction; D016212:Transforming Growth Factor beta / Q000235:genetics / Q000276:immunology*; D054392:Adipokines
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D000071838:Fibrillin-1; D000071837:Fibrillins; D008840:Microfilament Proteins; D007703:Peptidyl-Dipeptidase A; D016212:Transforming Growth Factor beta; C000605722:FBN1 protein, human; D054392:Adipokines; C488034:ACE protein, human