Pulmonary Vascular Pathology

Arterial-Lymphatic-Like Endothelial Cells Appear in Hereditary Hemorrhagic Telangiectasia 2 and Contribute to Vascular Leakage and Arteriovenous Malformations

Yang Yang, Xiuju Wu, Yan Zhao, Daoqin Zhang, Li Zhang, Xinjiang Cai, Jaden Ji, Zheng Jing, Kristina I. Boström, Yucheng YaoDavid Geffen School of Medicine and University of California. Stanford University.United States CirculationCirculation 2024; DOI: 10.1161/CIRCULATIONAHA.124.070925 AbstractBackground: Arteriovenous malformations (AVMs) are characteristic of hereditary hemorrhagic telangiectasia. Loss-of-function mutations in the activin receptor-like kinase 1 (Alk1) are linked […]

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ASXL1-related Bohring-Optiz Syndrome complicated by Persistent Neonatal Pulmonary Hypertension and Abnormal Alveoli Formation

Makoto Arioka, Shinji Nakamura, Katsufumi Nishioka, Kota Inoue, Yasuhiro Nakao, Yumi Miyai, Hirosuke Morita, Kosuke Koyano, Toshiki Takenouchi, Saneyuki Yasuda, Yoichi Chiba, Takashi Iwase, Masaki Ueno, Takashi KusakaKagawa University Hospital and Kagawa University. Keio University School of Medicine.Japan European Journal of Medical GeneticsEur J Med Genet 2024; DOI: 10.1016/j.ejmg.2024.104978 AbstractBohring-Opitz syndrome (BOS) is a rare disease

ASXL1-related Bohring-Optiz Syndrome complicated by Persistent Neonatal Pulmonary Hypertension and Abnormal Alveoli Formation Read More »

Nintedanib preserves lung growth and prevents pulmonary hypertension in a hyperoxia-induced lung injury model

Kathy L. Ding, Caroline Smith, Gregory Seedorf, Steven H. AbmanUniversity of Colorado School of Medicine.United States Pediatric ResearchPediatr Res 2024; DOI: 10.1038/s41390-024-03562-0 AbstractBackground: Bronchopulmonary dysplasia (BPD), the chronic lung disease associated with prematurity, is characterized by poor alveolar and vascular growth, interstitial fibrosis, and pulmonary hypertension (PH). Although multifactorial in origin, the pathophysiology of BPD is partly

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GCN2 kinase activation mediates pulmonary vascular remodeling and pulmonary arterial hypertension

Maggie M. Zhu, Jingbo Dai, Zhiyu Dai, Yi Peng, You-Yang ZhaoNorthwestern University Feinberg School of Medicine.United States Journal of Clinical Investigation InsightJCI Insight 2024; DOI: 10.1172/jci.insight.177926 AbstractPulmonary arterial hypertension (PAH) is characterized by progressive increase of pulmonary vascular resistance and remodeling that result in right heart failure. Recessive mutations of EIF2AK4 gene (encoding GCN2, General control

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G6pdN126D Variant Increases the Risk of Developing VEGFR (Vascular Endothelial Growth Factor Receptor) Blocker-Induced Pulmonary Vascular Disease

Christina Signoretti, Shun Matsumura, Samuel Fatehi, Melinee D’Silva, Rajamma Mathew, Francesca Cendali, Angelo D’Alessandro, S. M. Shafiqul Alam, Victor Garcia, Joseph M. Miano, Sachin A. GupteNew York Medical College. University of Colorado Anschutz Medical Campus. Medical College of Georgia at Augusta University. United States Journal of the American Medical AssociationJ Am Heart Assoc 2024; DOI: 10.1161/JAHA.123.035174 AbstractBackground: G6PD (glucose-6-phosphate-dehydrogenase) is a key enzyme in the glycolytic pathway and has been implicated in the pathogenesis of cancer

G6pdN126D Variant Increases the Risk of Developing VEGFR (Vascular Endothelial Growth Factor Receptor) Blocker-Induced Pulmonary Vascular Disease Read More »

Unraveling the Mfn2-Warburg effect nexus: a therapeutic strategy to combat pulmonary arterial hypertension arising from catch-up growth after IUGR

Lingling Yan, Xiaofei Luo, Chengcheng Hang, YuWang, Ziming Zhang, Shanshan Xu, Lizhong DuChildren’s Hospital and First Affiliated Hospital of Zhejiang University School of Medicine. China Respiratory ResearchRespir Res 2024; 25: DOI: 10.1186/s12931-024-02957-1 AbstractBackground: The interplay between intrauterine and early postnatal environments has been associated with an increased risk of cardiovascular diseases in adulthood, including pulmonary arterial hypertension

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Novel Tryptophan Hydroxylase Inhibitor TPT-001 Reverses PAH, Vascular Remodeling, and Proliferative-Proinflammatory Gene Expression

Ekaterina Legchenko, Philippe Chouvarine, Fatimunnisa Qadri, Edgar Specker, Marc Nazaré, Radoslaw Wesolowski, Susann Matthes, Michael Bader, Georg HansmannHannover Medical School. Max-Delbrück-Center for Molecular Medicine. Trypto Therapeutics GmbH. German Center for Cardiovascular Research. Charité Universitätsmedizin Berlin. University of Lübeck. Friedrich-Alexander-University Erlangen-Nürnberg. Germany Journal of the Amerian College of Cardiology Basic to Translational ScienceJACC Basi Transl Sci

Novel Tryptophan Hydroxylase Inhibitor TPT-001 Reverses PAH, Vascular Remodeling, and Proliferative-Proinflammatory Gene Expression Read More »

Integrative Multiomics in the Lung Reveals a Protective Role of Asporin in Pulmonary Arterial Hypertension

Jason Hong, Lejla Medzikovic, Wasila Sun, Brenda Wong, Grégoire Ruffenach, Christopher J. Rhodes, Adam Brownstein, Lloyd L. Liang, Laila Aryan, Min Li, Arjun Vadgama, Zeyneb Kurt, Tae-Hwi Schwantes-An, Elizabeth A. Mickler, Stefan Gräf, Mélanie Eyries, Katie A. Lutz, Michael W. Pauciulo, Richard C. Trembath, Frédéric Perros, David Montani, Nicholas W. Morrell, Florent Soubrier, Martin R.

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Animal models of pulmonary arterial hypertension associated with atrial septal defect

Li Wang, Ting Tang, Xiaochun Tian, Chang Peng, Shuqi WuGuizhou Children’s Hospital and Affiliated Hospital of Zunyi Medical University. China Scientific ReportsSci Rep 2024; 14: DOI: 10.1038/s41598-024-69002-5 AbstractPulmonary arterial hypertension (PAH) is a well-known complication of congenital heart disease (CHD). The lack of a satisfactory animal model for PAH associated with CHD (PAH-CHD) has limited progress

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General Capillary Endothelial Cells Undergo Reprogramming Into Arterial Endothelial Cells in Pulmonary Hypertension Through HIF-2α/Notch4 Pathway

Bin Liu, Dan Yi, Xiaomei Xia, Karina Ramirez, Hanqiu Zhao, Yanhong Cao, Ankit Tripathi, Ryan Dong, Anton Gao, Hongxu Ding, Shenfeng Qiu, Vladimir V. Kalinichenko,You-Yang Zhao, Michael B. Fallon, Zhiyu DaiUniversity of Arizona. Ann & Robert H. Lurie Children’s Hospital of Chicago and Northwestern University Feinberg School of Medicine.United States CirculationCirculation 2024; 150: 414-417DOI: 10.1161/CIRCULATIONAHA.123.067981 AbstractAbstract Not Available CategoryVascular Cell Biology and Mechanisms of Pulmonary Vascular DiseaseAnimal Models of Pulmonary Vascular Disease and TherapyPulmonary Vascular Pathology Age Focus: No

General Capillary Endothelial Cells Undergo Reprogramming Into Arterial Endothelial Cells in Pulmonary Hypertension Through HIF-2α/Notch4 Pathway Read More »

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