title: [Novel pharmacological strategies for aortic dilation in Marfan syndrome: from mouse models to human patients].
abstract: Marfan syndrome (MS) is a congenital disorder of the connective tissue characterized by aortic dilation with frequent progression to aortic aneurysms requiring surgical intervention. Although mutations in the fibrillin-1 (FBN1) gene have been recognized as the genetic cause of MS a long time ago, only recently deeper knowledge of the molecular mechanisms underlying fibrillin-1 biology and the crucial role of transforming growth factor-β and angiotensin II receptor type 1 antagonists have been elucidated. This review focuses on the most commonly used animal models to investigate the molecular mechanisms underlying MS, and on novel pharmacological strategies to reduce aortic dilation in MS.
authors.forename: Cinzia
authors.initials: C
authors.lastname: Perrino
authors.forename: Gabriele Giacomo
authors.initials: GG
authors.lastname: Schiattarella
authors.forename: Roberta
authors.initials: R
authors.lastname: Bottino
authors.forename: Fabio
authors.initials: F
authors.lastname: Magliulo
authors.forename: Marco
authors.initials: M
authors.lastname: Oliveti
authors.forename: Giovanni
authors.initials: G
authors.lastname: Esposito
authors.forename: Bruno
authors.initials: B
authors.lastname: Trimarco
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000494:pharmacology / Q000627:therapeutic use*; D047228:Angiotensin II Type 1 Receptor Blockers / Q000494:pharmacology / Q000627:therapeutic use*; D000818:Animals; D001014:Aortic Aneurysm / Q000188:drug therapy* / Q000235:genetics; D004359:Drug Therapy, Combination; D019317:Evidence-Based Medicine; D000071838:Fibrillin-1; D000071837:Fibrillins; D006801:Humans; D008382:Marfan Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics* / Q000503:physiopathology; D051379:Mice; D008840:Microfilament Proteins / Q000235:genetics; D009154:Mutation; D016212:Transforming Growth Factor beta / Q000235:genetics; D016896:Treatment Outcome; D054392:Adipokines
chemical_list: D000319:Adrenergic beta-Antagonists; D047228:Angiotensin II Type 1 Receptor Blockers; D000071838:Fibrillin-1; D000071837:Fibrillins; D008840:Microfilament Proteins; D016212:Transforming Growth Factor beta; C000605722:FBN1 protein, human; D054392:Adipokines; C000605724:Fbn1 protein, mouse