Impaired Bone Morphogenetic Protein Receptor II Signaling in a Transforming Growth Factor-β–Dependent Mouse Model of Pulmonary Hypertension and in Systemic Sclerosis

Prediction of Pulmonary Complications and Long-Term Survival in Systemic Sclerosis

(2014) Svetlana I. Nihtyanova et al.   Arthritis & Rheumatology

Inhibition of Overactive Transforming Growth Factor–β Signaling by Prostacyclin Analogs in Pulmonary Arterial Hypertension

(2013) Takeshi Ogo et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Endothelial Injury in a Transforming Growth Factor β-Dependent Mouse Model of Scleroderma Induces Pulmonary Arterial Hypertension

(2013) Emma C. Derrett-Smith et al.   ARTHRITIS AND RHEUMATISM

Chloroquine Prevents Progression of Experimental Pulmonary Hypertension via Inhibition of Autophagy and Lysosomal Bone Morphogenetic Protein Type II Receptor Degradation

(2013) Lu Long et al.   CIRCULATION RESEARCH

Epigenetic repression of bone morphogenetic protein receptor II expression in scleroderma

(2013) Yongqing Wang et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

Scleroderma pathogenesis: a pivotal role for fibroblasts as effector cells

(2013) Adrian J Gilbane et al.   ARTHRITIS RESEARCH & THERAPY

BMP type II receptor deficiency confers resistance to growth inhibition by TGF-β in pulmonary artery smooth muscle cells: role of proinflammatory cytokines

(2012) Rachel J. Davies et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Borderline Mean Pulmonary Artery Pressure in Patients With Systemic Sclerosis: Transpulmonary Gradient Predicts Risk of Developing Pulmonary Hypertension

(2012) Christopher J. Valerio et al.   ARTHRITIS AND RHEUMATISM

Targeting TGFβ superfamily ligand accessory proteins as novel therapeutics for chronic lung disorders

(2012) David C. Budd et al.   PHARMACOLOGY & THERAPEUTICS

Gut fibrosis with altered colonic contractility in a mouse model of scleroderma

(2012) N. M. Thoua et al.   RHEUMATOLOGY

A Novel Murine Model of Severe Pulmonary Arterial Hypertension

(2011) Loredana Ciuclan et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Smurf1 ubiquitin ligase causes downregulation of BMP receptors and is induced in monocrotaline and hypoxia models of pulmonary arterial hypertension

(2011) Koko Murakami et al.   EXPERIMENTAL BIOLOGY AND MEDICINE

Proteasomal inhibition after injury prevents fibrosis by modulating TGF-β1signalling

(2011) Gökhan M Mutlu et al.   THORAX

An Essential Role for Resident Fibroblasts in Experimental Lung Fibrosis Is Defined by Lineage-Specific Deletion of High-Affinity Type II Transforming Growth Factor β Receptor

(2010) Rachel K. Hoyles et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Identification of a Lysosomal Pathway Regulating Degradation of the Bone Morphogenetic Protein Receptor Type II

(2010) Hannah J. Durrington et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Prevalence of Pulmonary Hypertension in Systemic Sclerosis in European Caucasians and Metaanalysis of 5 Studies

(2010) J. AVOUAC et al.   JOURNAL OF RHEUMATOLOGY

Systemic vasculopathy with altered vasoreactivity in a transgenic mouse model of scleroderma

(2010) Emma C Derrett-Smith et al.   ARTHRITIS RESEARCH & THERAPY

Survival in pulmonary hypertension associated with the scleroderma spectrum of diseases: Impact of interstitial lung disease

(2009) Stephen C. Mathai et al.   ARTHRITIS AND RHEUMATISM

Altered Bone Morphogenetic Protein and Transforming Growth Factor-β Signaling in Rat Models of Pulmonary Hypertension

(2009) Lu Long et al.   CIRCULATION

Activin-Like Kinase 5 (ALK5) Mediates Abnormal Proliferation of Vascular Smooth Muscle Cells from Patients with Familial Pulmonary Arterial Hypertension and Is Involved in the Progression of Experimental Pulmonary Arterial Hypertension Induced by Monocrotaline

(2008) Matthew Thomas et al.   AMERICAN JOURNAL OF PATHOLOGY

Clinical Outcomes of Pulmonary Arterial Hypertension in Carriers ofBMPR2Mutation

(2008) Benjamin Sztrymf et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Fibroblast-specific perturbation of transforming growth factor β signaling provides insight into potential pathogenic mechanisms of scleroderma-associated lung fibrosis: Exaggerated response to alveolar epithelial injury in a novel mouse model

(2008) Rachel K. Hoyles et al.   ARTHRITIS AND RHEUMATISM