Circulating transforming growth factor-β in Marfan syndrome

P Matt, F Schoenhoff, J Habashi, T Holm, C Van Erp… - Circulation, 2009 - Am Heart Assoc
Background—Marfan syndrome (MFS) is caused by mutations in the fibrillin-1 gene and
dysregulation of transforming growth factor-β (TGF-β). Recent evidence suggests that …

Aortopathy in a mouse model of Marfan syndrome is not mediated by altered transforming growth factor β signaling

H Wei, JH Hu, SN Angelov, K Fox, J Yan… - Journal of the …, 2017 - Am Heart Assoc
Background Marfan syndrome (MFS) is caused by mutations in the gene encoding fibrillin‐1
(FBN 1); however, the mechanisms through which fibrillin‐1 deficiency causes MFS …

Circulating transforming growth factor-β as a prognostic biomarker in Marfan syndrome

R Franken, AW den Hartog, V de Waard… - International journal of …, 2013 - Elsevier
Abstract Background Patients with Marfan syndrome (MFS) are at risk for cardiovascular
disease. Marfan associated mutations in the FBN1 gene lead to increased transforming …

[HTML][HTML] Recent advances in understanding Marfan syndrome: should we now treat surgical patients with losartan?

P Matt, J Habashi, T Carrel, DE Cameron… - The Journal of thoracic …, 2008 - Elsevier
OBJECTIVE: Marfan syndrome is a systemic connective tissue disorder caused by mutations
in the fibrillin-1 gene. It was originally believed that Marfan syndrome results exclusively …

Beneficial Outcome of Losartan Therapy Depends on Type of FBN1 Mutation in Marfan Syndrome

R Franken, AW den Hartog, T Radonic… - Circulation …, 2015 - Am Heart Assoc
Background—It has been shown that losartan reduces aortic dilatation in patients with
Marfan syndrome. However, treatment response is highly variable. This study investigates …

Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome

JP Habashi, DP Judge, TM Holm, RD Cohn, BL Loeys… - Science, 2006 - science.org
Aortic aneurysm and dissection are manifestations of Marfan syndrome (MFS), a disorder
caused by mutations in the gene that encodes fibrillin-1. Selected manifestations of MFS …

Therapy of Marfan syndrome

DP Judge, HC Dietz - Annu. Rev. Med., 2008 - annualreviews.org
Marfan syndrome is a common inherited disorder of connective tissue caused by deficiency
of the matrix protein fibrillin-1. Effective surgical therapy for the most life-threatening …

Pathophysiology and management of cardiovascular manifestations in Marfan and Loeys–Dietz syndromes

N Takeda, H Yagi, H Hara, T Fujiwara… - International heart …, 2016 - jstage.jst.go.jp
Marfan syndrome (MFS) is an autosomal dominant heritable disorder of connective tissue
that affects the cardiovascular, skeletal, ocular, pulmonary, and nervous systems and is …

Comparison of Clinical Presentations and Outcomes Between Patients With TGFBR2 and FBN1 Mutations in Marfan Syndrome and Related Disorders

D Attias, C Stheneur, C Roy, G Collod-Béroud… - Circulation, 2009 - Am Heart Assoc
Background—TGFBR2 mutations were recognized recently among patients with a Marfan-
like phenotype. The associated clinical and prognostic spectra remain unclear. Methods and …

[HTML][HTML] Marfan syndrome revisited: From genetics to clinical practice

SG Coelho, AG Almeida - Revista Portuguesa de Cardiologia (English …, 2020 - Elsevier
Marfan syndrome is an autosomal dominant connective tissue disease with an estimated
incidence of 1 in 5000 individuals. In 90% of cases it is caused by mutations in the gene for …