Abnormal Trafficking of Endogenously Expressed BMPR2 Mutant Allelic Products in Patients with Heritable Pulmonary Arterial Hypertension

Correction of NonsenseBMPR2andSMAD9Mutations by Ataluren in Pulmonary Arterial Hypertension

(2013) Kylie M. Drake et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

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

The Unfolded Protein Response and Chemical Chaperones Reduce Protein Misfolding and Colitis in Mice

(2013) Stewart Siyan Cao et al.   GASTROENTEROLOGY

Doxycycline plus tauroursodeoxycholic acid for transthyretin amyloidosis: a phase II study

(2012) Laura Obici et al.   AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS

A Host–Guest Relationship in Bone Morphogenetic Protein Receptor-II Defines Specificity in Ligand–Receptor Recognition

(2012) Lee-Chuan C. Yeh et al.   BIOCHEMISTRY

Promiscuity and specificity in BMP receptor activation

(2012) Thomas D. Mueller et al.   FEBS LETTERS

Functional rescue of mutant ABCA1 proteins by sodium 4-phenylbutyrate

(2012) Brie Sorrenson et al.   JOURNAL OF LIPID RESEARCH

Disulfide Bond Formation in the Mammalian Endoplasmic Reticulum

(2012) N. J. Bulleid   Cold Spring Harbor Perspectives in Biology

Defective trafficking of cone photoreceptor CNG channels induces the unfolded protein response and ER-stress-associated cell death

(2011) Deborah L. Duricka et al.   BIOCHEMICAL JOURNAL

The chemical chaperones tauroursodeoxycholic and 4-phenylbutyric acid accelerate thyroid hormone activation and energy expenditure

(2011) Wagner S. da-Silva et al.   FEBS LETTERS

Idiopathic and Heritable PAH Perturb Common Molecular Pathways, Correlated with Increased MSX1 Expression

(2011) Eric D. Austin et al.   Pulmonary Circulation

ID family protein expression and regulation in hypoxic pulmonary hypertension

(2010) Jonathan W. Lowery et al.   AMERICAN JOURNAL OF PHYSIOLOGY-REGULATORY INTEGRATIVE AND COMPARATIVE PHYSIOLOGY

Bone morphogenetic protein receptor II regulates pulmonary artery endothelial cell barrier function

(2010) V. J. Burton et al.   BLOOD

Transcripts from a novelBMPR2termination mutation escape nonsense mediated decay by downstream translation re-initiation: implications for treating pulmonary hypertension

(2010) R Hamid et al.   CLINICAL GENETICS

BMP signaling in vascular development and disease

(2010) Jonathan W. Lowery et al.   CYTOKINE & GROWTH FACTOR REVIEWS

Tauroursodeoxycholic Acid Attenuates Lipid Accumulation in Endoplasmic Reticulum–Stressed Macrophages

(2010) Yinan Hua et al.   JOURNAL OF CARDIOVASCULAR PHARMACOLOGY

Bmp2 and Bmp4 exert opposing effects in hypoxic pulmonary hypertension

(2009) Lynda Anderson et al.   AMERICAN JOURNAL OF PHYSIOLOGY-REGULATORY INTEGRATIVE AND COMPARATIVE PHYSIOLOGY

Recent advances in BMP receptor signaling

(2009) Christina Sieber et al.   CYTOKINE & GROWTH FACTOR REVIEWS

Penetrance of pulmonary arterial hypertension is modulated by the expression of normalBMPR2allele

(2009) Rizwan Hamid et al.   HUMAN MUTATION

Genetics of Pulmonary Arterial Hypertension

(2009) Eric Austin et al.   SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE

Inflammation, endothelial injury, and persistent pulmonary hypertension in heterozygous BMPR2-mutant mice

(2008) Yanli Song et al.   AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY

Gene expression in BMPR2 mutation carriers with and without evidence of Pulmonary Arterial Hypertension suggests pathways relevant to disease penetrance

(2008) James West et al.   BMC Medical Genomics

Genetic Ablation of the Bmpr2 Gene in Pulmonary Endothelium Is Sufficient to Predispose to Pulmonary Arterial Hypertension

(2008) Kwon-Ho Hong et al.   CIRCULATION

Failure of bone morphogenetic protein receptor trafficking in pulmonary arterial hypertension: potential for rescue

(2008) A. Sobolewski et al.   HUMAN MOLECULAR GENETICS

Characterization of Ligand-Binding Properties of the Human BMP Type II Receptor Extracellular Domain

(2008) Huiran Yin et al.   JOURNAL OF MOLECULAR BIOLOGY

Proinflammatory Effect of Sodium 4-Phenylbutyrate in  F508-Cystic Fibrosis Transmembrane Conductance Regulator Lung Epithelial Cells: Involvement of Extracellular Signal-Regulated Protein Kinase 1/2 and c-Jun-NH2-Terminal Kinase Signaling

(2008) T. Roque et al.   JOURNAL OF PHARMACOLOGY AND EXPERIMENTAL THERAPEUTICS

Increased susceptibility to hypoxic pulmonary hypertension in Bmpr2 mutant mice is associated with endothelial dysfunction in the pulmonary vasculature

(2007) David B. Frank et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY