Function of Ltbp-4L and fibulin-4 in survival and elastogenesis in mice

Differences in genetic signaling, and not mechanical properties of the wall, are linked to ascending aortic aneurysms in fibulin-4 knockout mice

(2015) Jungsil Kim et al.   AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY

Arterial tortuosity in genetic arteriopathies

(2015) Shaine A. Morris   CURRENT OPINION IN CARDIOLOGY

Modeling autosomal recessive cutis laxa type 1C in mice reveals distinct functions for Ltbp-4 isoforms

(2015) I. Bultmann-Mellin et al.   Disease Models & Mechanisms

Loss of fibulin-4 disrupts collagen synthesis and maturation: implications for pathology resulting fromEFEMP2mutations

(2015) Christina L. Papke et al.   HUMAN MOLECULAR GENETICS

Increased aortic tortuosity indicates a more severe aortic phenotype in adults with Marfan syndrome

(2015) Romy Franken et al.   INTERNATIONAL JOURNAL OF CARDIOLOGY

Latent TGF-β-binding proteins

(2015) Ian B. Robertson et al.   MATRIX BIOLOGY

Function of Latent TGFβ Binding Protein 4 and Fibulin 5 in Elastogenesis and Lung Development

(2014) Branka Dabovic et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Extracellular Matrix Defects in Aneurysmal Fibulin-4 Mice Predispose to Lung Emphysema

(2014) Natasja W. M. Ramnath et al.   PLoS One

Recurrent Gain-of-Function Mutation in PRKG1 Causes Thoracic Aortic Aneurysms and Acute Aortic Dissections

(2013) Dong-chuan Guo et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Severe aortopathy due to fibulin-4 deficiency: molecular insights, surgical strategy, and a review of the literature

(2013) Camden Hebson et al.   EUROPEAN JOURNAL OF PEDIATRICS

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

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

Latent Transforming Growth Factor β-Binding Protein 4 Is Downregulated in Esophageal Cancer via Promoter Methylation

(2013) Insa Bultmann et al.   PLoS One

Latent TGF-  binding protein 4 promotes elastic fiber assembly by interacting with fibulin-5

(2013) K. Noda et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Predicting the Risk for Acute Type B Aortic Dissection in Hypertensive Patients Using Anatomic Variables

(2013) Aditya S. Shirali et al.   JACC-Cardiovascular Imaging

Comprehensive Clinical and Molecular Analysis of 12 Families with Type 1 Recessive Cutis Laxa

(2012) Bert Callewaert et al.   HUMAN MUTATION

Aggressive Cardiovascular Phenotype of Aneurysms-Osteoarthritis Syndrome Caused by Pathogenic SMAD3 Variants

(2012) Denise van der Linde et al.   JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

Loss-of-function mutations in TGFB2 cause a syndromic presentation of thoracic aortic aneurysm

(2012) Mark E Lindsay et al.   NATURE GENETICS

Increased Vertebral Artery Tortuosity Index Is Associated With Adverse Outcomes in Children and Young Adults With Connective Tissue Disorders

(2011) Shaine A. Morris et al.   CIRCULATION

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

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

Unraveling the mechanism of elastic fiber assembly: The roles of short fibulins

(2010) Hiromi Yanagisawa et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Independent regulation of short and long forms of latent TGF-β binding protein (LTBP)-4 in cultured fibroblasts and human tissues

(2010) Anna K. Kantola et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Mutations in LTBP4 Cause a Syndrome of Impaired Pulmonary, Gastrointestinal, Genitourinary, Musculoskeletal, and Dermal Development

(2009) Zsolt Urban et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Assessment of the association between genetic polymorphisms in transforming growth factor beta, and its binding protein (LTBP), and the presence, and expansion, of Abdominal Aortic Aneurysm

(2009) Andrew R. Thompson et al.   ATHEROSCLEROSIS

Reduced Vessel Elasticity Alters Cardiovascular Structure and Function in Newborn Mice

(2009) Jessica E. Wagenseil et al.   CIRCULATION RESEARCH

Transient tropoelastin nanoparticles are early-stage intermediates in the coacervation of human tropoelastin whose aggregation is facilitated by heparan sulfate and heparin decasaccharides

(2009) Yidong Tu et al.   MATRIX BIOLOGY

Mutations in the SLC2A10 gene cause arterial abnormalities in mice

(2008) C.-H. Cheng et al.   CARDIOVASCULAR RESEARCH

Dual functions for LTBP in lung development: LTBP-4 independently modulates elastogenesis and TGF-β activity

(2008) Branka Dabovic et al.   JOURNAL OF CELLULAR PHYSIOLOGY