Human liver single nuclear RNA sequencing implicates BMPR2, GDF15, arginine, and estrogen in portopulmonary hypertension

A Jose, JM Elwing, SM Kawut, MW Pauciulo… - Communications …, 2023 - nature.com
Portopulmonary hypertension (PoPH) is a type of pulmonary vascular disease due to portal
hypertension that exhibits high morbidity and mortality. The mechanisms driving disease are …

Bone morphogenetic protein 9 is a mechanistic biomarker of portopulmonary hypertension

I Nikolic, LM Yung, P Yang, R Malhotra… - American journal of …, 2019 - atsjournals.org
Rationale: BMP9 (bone morphogenetic protein 9) is a circulating endothelial quiescence
factor with protective effects in pulmonary arterial hypertension (PAH). Loss-of-function …

Disrupted BMP-9 signaling impairs pulmonary vascular integrity in hepatopulmonary syndrome

F Robert, MC Certain, A Baron, R Thuillet… - American Journal of …, 2024 - atsjournals.org
Rationale: Hepatopulmonary syndrome (HPS) is a severe complication of liver diseases
characterized by abnormal dilation of pulmonary vessels, resulting in impaired oxygenation …

BMPR2 expression is suppressed by signaling through the estrogen receptor

ED Austin, R Hamid, AR Hemnes, JE Loyd… - Biology of sex …, 2012 - Springer
Background Studies in multiple organ systems have shown cross-talk between signaling
through the bone morphogenetic protein receptor type 2 (BMPR2) and estrogen pathways …

PTPN1 deficiency modulates BMPR2 signaling and induces endothelial dysfunction in Pulmonary Arterial Hypertension

MK Ali, X Tian, L Zhao, K Schimmel, CJ Rhodes… - Cells, 2023 - mdpi.com
Bone morphogenic protein receptor 2 (BMPR2) expression and signaling are impaired in
pulmonary arterial hypertension (PAH). How BMPR2 signaling is decreased in PAH is …

Different cardiovascular and pulmonary phenotypes for single-and double-knock-out mice deficient in BMP9 and BMP10

C Bouvard, L Tu, M Rossi… - Cardiovascular …, 2022 - academic.oup.com
Aims BMP9 and BMP10 mutations were recently identified in patients with pulmonary
arterial hypertension, but their specific roles in the pathogenesis of the disease are still …

Bone morphogenetic protein signalling in pulmonary arterial hypertension: revisiting the BMPRII connection

W Li, K Quigley - Biochemical Society Transactions, 2024 - portlandpress.com
Pulmonary arterial hypertension (PAH) is a rare and life-threatening vascular disorder,
characterised by abnormal remodelling of the pulmonary vessels and elevated pulmonary …

Genetic risk factors for portopulmonary hypertension in patients with advanced liver disease

KE Roberts, MB Fallon, MJ Krowka… - American journal of …, 2009 - atsjournals.org
Rationale: Portopulmonary hypertension (PPHTN) occurs in 6% of liver transplant
candidates. The pathogenesis of this complication of portal hypertension is poorly …

At the X-roads of sex and genetics in pulmonary arterial hypertension

MM Cirulis, MW Dodson, LM Brown, SM Brown… - Genes, 2020 - mdpi.com
Group 1 pulmonary hypertension (pulmonary arterial hypertension; PAH) is a rare disease
characterized by remodeling of the small pulmonary arteries leading to progressive …

Consequences of BMPR2 deficiency in the pulmonary vasculature and beyond: contributions to pulmonary arterial hypertension

A Andruska, E Spiekerkoetter - International journal of molecular sciences, 2018 - mdpi.com
Since its association with familial pulmonary arterial hypertension (PAH) in 2000, Bone
Morphogenetic Protein Receptor II (BMPR2) and its related signaling pathway have become …