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Nature Communications, ISSN 2041-1723, 04/2018, Volume 9, Issue 1, pp. 1416 - 16
Pulmonary arterial hypertension (PAH) is a rare disorder with a poor prognosis. Deleterious variation within components of the transforming growth factor-beta... 
a_open_article_in_open_journal | SOX17 | GENE | ANGIOGENESIS | CRYSTAL-STRUCTURE | MULTIDISCIPLINARY SCIENCES | BMP9 | ASSOCIATION | EXPRESSION | NONSENSE MUTATION | BETA-RECEPTOR | CAVEOLIN-1 | Prognosis | Humans | Male | Familial Primary Pulmonary Hypertension - diagnosis | SOXF Transcription Factors - metabolism | Aquaporin 1 - metabolism | Case-Control Studies | Familial Primary Pulmonary Hypertension - metabolism | Membrane Transport Proteins - genetics | Base Sequence | HEK293 Cells | Adult | Female | Growth Differentiation Factors - genetics | Membrane Transport Proteins - metabolism | Familial Primary Pulmonary Hypertension - pathology | Genetic Predisposition to Disease | Bone Morphogenetic Protein Receptors, Type II - genetics | Signal Transduction | Gene Expression Regulation | Adenosine Triphosphatases - metabolism | Models, Molecular | Familial Primary Pulmonary Hypertension - genetics | Bone Morphogenetic Protein Receptors, Type II - metabolism | Membrane Transport Proteins - chemistry | Whole Genome Sequencing | Aquaporin 1 - chemistry | Aquaporin 1 - genetics | Growth Differentiation Factors - metabolism | Transforming Growth Factor beta - genetics | Adenosine Triphosphatases - chemistry | Adenosine Triphosphatases - genetics | Mutation | SOXF Transcription Factors - genetics | Transforming Growth Factor beta - metabolism | SOXF Transcription Factors - chemistry | Growth Differentiation Factors - chemistry | Hypertension | Bone morphogenetic protein receptor type II | Secretion | Transforming growth factor | Genes | Genomes | Heritability | Aquaporin 1 | Gene sequencing
Journal Article
Journal Article
Journal Article
Cardiovascular Research, ISSN 0008-6363, 06/2016, Volume 110, Issue 3, pp. 319 - 330
Journal Article
Lancet Respiratory Medicine, The, ISSN 2213-2600, 2016, Volume 4, Issue 2, pp. 129 - 137
Journal Article
Journal of Clinical Investigation, ISSN 0021-9738, 05/2018, Volume 128, Issue 5, pp. 1956 - 1970
Pulmonary arterial hypertension (PAH) is characterized by a progressive accumulation of pulmonary artery smooth muscle cells (PA-SMCs) in pulmonary arterioles... 
MEDICINE, RESEARCH & EXPERIMENTAL | FIBROBLAST GROWTH FACTOR-2 | INTERLEUKIN-6 RECEPTOR | OVEREXPRESSION | SMOOTH-MUSCLE-CELLS | MICE | CYTOKINE | BMPR-II | CONTRIBUTES | IL-6 RECEPTOR | BETA | Familial Primary Pulmonary Hypertension - pathology | Up-Regulation | Vascular Remodeling | Muscle, Smooth, Vascular - metabolism | Humans | Myocytes, Smooth Muscle - pathology | Rats | Familial Primary Pulmonary Hypertension - genetics | Mice, Transgenic | Pulmonary Artery - physiopathology | Signal Transduction - genetics | Muscle, Smooth, Vascular - physiopathology | Familial Primary Pulmonary Hypertension - metabolism | Familial Primary Pulmonary Hypertension - physiopathology | Arterioles - pathology | Muscle, Smooth, Vascular - pathology | Pulmonary Artery - metabolism | Animals | Arterioles - metabolism | Arterioles - physiopathology | Cytokine Receptor gp130 - biosynthesis | Mice | Myocytes, Smooth Muscle - metabolism | Pulmonary Artery - pathology | Care and treatment | Cytokine receptors | Physiological aspects | Development and progression | Health aspects | Pulmonary artery | Pulmonary hypertension | Interleukin-6 | Cytochrome | Hypertension | Animal models | Cytokines | Arterioles | Pathogenesis | Pulmonary arteries | Smooth muscle | Cardiovascular disease | Interleukin 6 | Proteins | Signal transduction | Interleukin 6 receptors | Rodents | Medical prognosis | Hypoxia | Heart diseases | Growth factors | Glycoprotein gp130 | Veins & arteries
Journal Article
Circulation, ISSN 0009-7322, 10/2016, Volume 134, Issue 15, pp. 1105 - 1121
Journal Article
Science Translational Medicine, ISSN 1946-6234, 10/2017, Volume 9, Issue 413, p. eaao4583
Journal Article
Arteriosclerosis, Thrombosis, and Vascular Biology, ISSN 1079-5642, 06/2017, Volume 37, Issue 8, pp. 1559 - 1569
OBJECTIVE—We determined in patients with pulmonary arterial (PA) hypertension (PAH) whether in addition to increased production of elastase by PA smooth muscle... 
smooth muscle cells | TGF-beta-1 | fibroblasts | BMPR2 receptor | hypertension, pulmonary | fibrillin-1 | elastin | BMPR2 | ENDOTHELIAL DYSFUNCTION | FIBRILLIN | IN-VITRO | GENE | MARFAN-SYNDROME | PERIPHERAL VASCULAR DISEASE | SMOOTH-MUSCLE-CELLS | MICE | MUTATIONS | HEMATOLOGY | EXPRESSION | Vascular Remodeling | Humans | Myocytes, Smooth Muscle - pathology | Hypertension, Pulmonary - physiopathology | Mice, 129 Strain | Case-Control Studies | Familial Primary Pulmonary Hypertension - metabolism | Familial Primary Pulmonary Hypertension - physiopathology | Elastic Tissue - metabolism | Pulmonary Artery - metabolism | Transfection | RNA Interference | Bone Morphogenetic Protein Receptors, Type I - deficiency | Fibrillin-1 - metabolism | Fibrillin-1 - genetics | Myocytes, Smooth Muscle - drug effects | Myocytes, Smooth Muscle - metabolism | Disease Models, Animal | Fibroblasts - metabolism | Familial Primary Pulmonary Hypertension - pathology | Elastin - metabolism | Genetic Predisposition to Disease | Bone Morphogenetic Protein Receptors, Type II - genetics | Mice, Inbred C57BL | Bone Morphogenetic Protein Receptors, Type II - deficiency | Cells, Cultured | Bone Morphogenetic Protein Receptors, Type I - genetics | Familial Primary Pulmonary Hypertension - genetics | Hypertension, Pulmonary - genetics | Hypertension, Pulmonary - metabolism | Pulmonary Artery - physiopathology | Elastic Tissue - physiopathology | Pulmonary Artery - drug effects | Fibroblasts - pathology | Bone Morphogenetic Protein 4 - pharmacology | Bone Morphogenetic Protein Receptors, Type II - metabolism | Mice, Knockout | Elastic Tissue - pathology | Transforming Growth Factor beta - pharmacology | Phenotype | Animals | Elastin - genetics | Fibroblasts - drug effects | Mutation | Hypertension, Pulmonary - pathology | Pulmonary Artery - pathology
Journal Article
Lancet Respiratory Medicine, The, ISSN 2213-2600, 2017, Volume 5, Issue 2, pp. 125 - 134
Journal Article