Fibulin-4 deficiency increases TGF-β signalling in aortic smooth muscle cells due to elevated TGF-β2 levels

Longer Term Survival of a Child With Autosomal Recessive Cutis Laxa Due to a Mutation inFBLN4

(2013) Sarah L. Sawyer et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

Aortic Remodeling After Transverse Aortic Constriction in Mice Is Attenuated With AT 1 Receptor Blockade

(2013) Shao-Qing Kuang et al.   ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY

Angiotensin II–dependent TGF-β signaling contributes to Loeys-Dietz syndrome vascular pathogenesis

(2013) Elena M. Gallo et al.   JOURNAL OF CLINICAL INVESTIGATION

TGF-β signaling in tissue fibrosis: Redox controls, target genes and therapeutic opportunities

(2012) Rohan Samarakoon et al.   CELLULAR SIGNALLING

TGFβRIIb Mutations Trigger Aortic Aneurysm Pathogenesis by Altering Transforming Growth Factor β2 Signal Transduction

(2012) Katharine J. Bee et al.   Circulation-Cardiovascular Genetics

Thoracic aortic-aneurysm and dissection in association with significant mitral valve disease caused by mutations in TGFB2

(2012) Marjolijn Renard et al.   INTERNATIONAL JOURNAL OF CARDIOLOGY

Interaction with colon cancer cells hyperactivates TGF-β signaling in cancer-associated fibroblasts

(2012) L J A C Hawinkels et al.   ONCOGENE

Characterization of a distinct lethal arteriopathy syndrome in twenty-two infants associated with an identical, novel mutation in FBLN4 gene, confirms fibulin-4 as a critical determinant of human vascular elastogenesis

(2012) Mahesh Kappanayil et al.   Orphanet Journal of Rare Diseases

The role of TGF-β in the pathogenesis of primary open-angle glaucoma

(2011) Rudolf Fuchshofer et al.   CELL AND TISSUE RESEARCH

Exploring anti-TGF-β therapies in cancer and fibrosis

(2011) Lukas J. A. C. Hawinkels et al.   GROWTH FACTORS

Novel MYH11 and ACTA2 mutations reveal a role for enhanced TGFβ signaling in FTAAD

(2011) Marjolijn Renard et al.   INTERNATIONAL JOURNAL OF CARDIOLOGY

Lessons on the pathogenesis of aneurysm from heritable conditions

(2011) Mark E. Lindsay et al.   NATURE

Mutations in SMAD3 cause a syndromic form of aortic aneurysms and dissections with early-onset osteoarthritis

(2011) Ingrid M B H van de Laar et al.   NATURE GENETICS

Lethal Osteogenesis Imperfecta–Like Condition with Cutis Laxa and Arterial Tortuosity in MZ Twins Due to a Homozygous Fibulin-4 Mutation

(2011) Lance K. Erickson et al.   PEDIATRIC AND DEVELOPMENTAL PATHOLOGY

Impaired Vascular Contractility and Aortic Wall Degeneration in Fibulin-4 Deficient Mice: Effect of Angiotensin II Type 1 (AT1) Receptor Blockade

(2011) Els Moltzer et al.   PLoS One

Mutations in Myosin Light Chain Kinase Cause Familial Aortic Dissections

(2010) Li Wang et al.   AMERICAN JOURNAL OF HUMAN GENETICS

TGFBR2 mutations alter smooth muscle cell phenotype and predispose to thoracic aortic aneurysms and dissections

(2010) Sakiko Inamoto et al.   CARDIOVASCULAR RESEARCH

Multimodality Imaging Reveals a Gradual Increase in Matrix Metalloproteinase Activity at Aneurysmal Lesions in Live Fibulin-4 Mice

(2010) Eric L. Kaijzel et al.   Circulation-Cardiovascular Imaging

Altered TGFβ signaling and cardiovascular manifestations in patients with autosomal recessive cutis laxa type I caused by fibulin-4 deficiency

(2010) Marjolijn Renard et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

Assembly of fibrillin microfibrils governs extracellular deposition of latent TGF 

(2010) T. Massam-Wu et al.   JOURNAL OF CELL SCIENCE

TGF-β through Smad3 signaling stimulates vascular smooth muscle cell proliferation and neointimal formation

(2009) Shirling Tsai et al.   AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY

Fibulin-4 regulates expression of the tropoelastin gene and consequent elastic-fibre formation by human fibroblasts

(2009) Qiuyun Chen et al.   BIOCHEMICAL JOURNAL

Active TGF-β1 correlates with myofibroblasts and malignancy in the colorectal adenoma-carcinoma sequence

(2009) Lukas J. A. C. Hawinkels et al.   CANCER SCIENCE

Fibulin-4 Deficiency Results in Ascending Aortic Aneurysms

(2009) Jianbin Huang et al.   CIRCULATION RESEARCH

Fibulin-4 conducts proper elastogenesis via interaction with cross-linking enzyme lysyl oxidase

(2009) Masahito Horiguchi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

VEGF release by MMP-9 mediated heparan sulphate cleavage induces colorectal cancer angiogenesis

(2008) Lukas J.A.C. Hawinkels et al.   EUROPEAN JOURNAL OF CANCER

Angiotensin II Blockade and Aortic-Root Dilation in Marfan's Syndrome

(2008) Benjamin S. Brooke et al.   NEW ENGLAND JOURNAL OF MEDICINE