scholarly journals Characterization of a caveolin-1 mutation associated with both pulmonary arterial hypertension and congenital generalized lipodystrophy

Traffic ◽  
2016 ◽  
Vol 17 (12) ◽  
pp. 1297-1312 ◽  
Author(s):  
Bing Han ◽  
Courtney A. Copeland ◽  
Yumeko Kawano ◽  
Erika Berman Rosenzweig ◽  
Eric D. Austin ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Caveolin 1 ◽  
Congenital Generalized Lipodystrophy ◽  
Pulmonary Arterial

Model Characterization of Pulmonary Arterial Hypertension: Analysis of Lung Pathology with Respect to Human Disease

2020 ◽  
Author(s):  
◽  
Eptisam lambu
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Human Disease ◽  
Vascular Remodeling ◽  
Vascular Wall ◽  
Lung Pathology ◽  
Pulmonary Arterial ◽  
Lung Pathogenesis ◽  
Arterial Endothelial Cells

Pulmonary arterial hypertension (PAH) is a rare multifactorial disease characterized by abnormal high blood pressure in the pulmonary artery, or increased pulmonary vascular resistance (PVR), caused by obstruction in the small arteries of the lung. Increased PVR is also thought to be caused by abnormal vascular remodeling, due to thickening of the pulmonary vascular wall resulting from significant hypertrophy of pulmonary arterial smooth-muscle cells (PASMCs) and increased proliferation/impaired apoptosis of pulmonary arterial endothelial cells (PAECs). Herein, we investigated the mechanisms and explored molecular pathways mediating the lung pathogenesis in two PAH rat models: Monocrotaline (MCT) and Sugen5416/Hypoxia (SuHx). We analyzed these disease models to determine where the vasculature shows the most severe PAH pathology and which model best recapitulates the human disease. We investigated the role vascular remodeling, hypoxia, cell proliferation, apoptosis, DNA damage and inflammation play in the pathogenesis of PAH. Neither model recapitulated all features of the human disease, however each model presented with some of the pathology seen in PAH patients.

scholarly journals Characterization of First-Time Hospitalizations in Patients With Newly Diagnosed Pulmonary Arterial Hypertension in the REVEAL Registry

CHEST Journal ◽  
2014 ◽  
Vol 146 (5) ◽  
pp. 1263-1273 ◽  
Author(s):  
Charles D Burger ◽  
Pamela K Long ◽  
Monarch R Shah ◽  
Michael D McGoon ◽  
Dave P Miller ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Newly Diagnosed ◽  
Pulmonary Arterial ◽  
First Time

Characterization of Pulmonary Arterial Hypertension Patients Walking More Than 450 m in 6 Min at Diagnosis

CHEST Journal ◽  
2010 ◽  
Vol 137 (6) ◽  
pp. 1297-1303 ◽  
Author(s):  
Bruno Degano ◽  
Olivier Sitbon ◽  
Laurent Savale ◽  
Gilles Garcia ◽  
Dermot S. O'Callaghan ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pulmonary Arterial

scholarly journals Characterization of Connective Tissue Disease-Associated Pulmonary Arterial Hypertension From REVEAL

CHEST Journal ◽  
2010 ◽  
Vol 138 (6) ◽  
pp. 1383-1394 ◽  
Author(s):  
Lorinda Chung ◽  
Juliana Liu ◽  
Lori Parsons ◽  
Paul M. Hassoun ◽  
Michael McGoon ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Connective Tissue Disease ◽  
Pulmonary Arterial

scholarly journals Molecular and functional characterization of the BMPR2 gene in Pulmonary Arterial Hypertension

2017 ◽  
Vol 7 (1) ◽  
Author(s):  
Guillermo Pousada ◽  
Vincenzo Lupo ◽  
Sheila Cástro-Sánchez ◽  
María Álvarez-Satta ◽  
Ana Sánchez-Monteagudo ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Functional Characterization ◽  
Pulmonary Arterial
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