ArticleScience (New York, N.Y.)2025
Lysosomal dysfunction and inflammatory sterol metabolism in pulmonary arterial hypertension.
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Article in Science (New York, N.Y.), 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers.
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33citing papers in PubMed
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Who cites it
33 citing papers in PubMed.
- Transcriptomic characteristics of plasma from Chinese ethnic minority patients with major depressive disorder.Translational psychiatry · 2026Article
- Lysosome-derived methylated arginine is a signalling metabolite controlling the lipidome.Nature cell biology · 2026Article
- Metabolic Markers as Potential Risk Factors in Pulmonary Hypertension.Pulmonary circulation · 2026Article
- Metabolomic Network Analysis Reveals Reorganization of Lipid and Steroid Programs Linked to Right Ventricular-Pulmonary Vascular Function in Pulmonary Hypertension.bioRxiv : the preprint server for biology · 2026Article
- Exploring the Lung-Liver Axis in Pulmonary Arterial Hypertension.Comprehensive Physiology · 2026Article
- Therapeutic Effects of Active Compounds fromACS omega · 2026Article
- Response to Kawamoto et al.EMBO reports · 2026Article
- Network Toxicology Reveals the Mechanisms of the Plasticizer Metabolite MECPP in Metabolic Diseases.International journal of molecular sciences · 2026Article
- Multi-Omics for Mothers and Infants (MOMI) Consortium: a global initiative to study adverse pregnancy outcomes.Journal of global health · 2026Article
- The Importance of Liver-Lung Communication in Pulmonary Vascular Diseases.Comprehensive Physiology · 2026Review
- Advances in the pathogenesis and clinical management of pulmonary hypertension.Medical review (2021) · 2026Review
- Metabolic Dysregulation of 27-Hydroxycholesterol Sensitizes Proximal Tubular Epithelial Cells to Ferroptosis in Ischemic Acute Kidney Injury.Journal of the American Society of Nephrology : JASN · 2026Article
- The lysosomal LAMTOR-Rag complex functions as a checkpoint for antiviral interferon production.The EMBO journal · 2026Article
- Microbiome-Metabolome Axis in BALF Reveals Novel Diagnostic Biomarkers for Congenital Heart Disease-Associated Pulmonary Arterial Hypertension.Journal of cardiovascular development and disease · 2026Article
- Endothelial ADAR1 Deficit Induces the NOCT-IRF7 Axis in Pulmonary Hypertension.Circulation research · 2026Article
- Defect in lysosomal enzyme trafficking and sorting is associated with irreversibility of pulmonary arterial hypertension.Frontiers in cardiovascular medicine · 2026Article
- Emerging insights of bile acids in cardiovascular physiology and disease: from molecular mechanisms to therapeutic potential.Frontiers in pharmacology · 2026Review
- Proteomic signatures of carotid plaque vulnerability: Proteolysis, inflammation, metabolic reprogramming, and lipid dysregulation.JVS-vascular science · 2026Article
- Roles of Lipid Metabolism in Pulmonary Hypertension: Friend or Foe?Biomolecules · 2025Review
- Lysosomal control of proteostasis and reproductive capacity by conserved LMD-3 protein inScience advances · 2025Article
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Authors and funding
51 authors.
Lloyd D HarveyMedical Scientist Training Program, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0000-0001-5934-9934
Mona AlotaibiDivision of Pulmonary and Critical Care Medicine, University of California San Diego, La Jolla, CA, USA.ORCID 0000-0001-8492-9930
Yi-Yin TaiCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0000-0003-3674-0783
Ying TangCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.
Hee-Jung J KimCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0009-0004-3353-0777
Neil J KellyCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0000-0003-0455-5348
Wei SunCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0000-0002-5255-1213
Chen-Shan C WoodcockCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0000-0003-4291-1788
Sanya ArshadCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.
Miranda K CulleyMedical Scientist Training Program, University of Pittsburgh, Pittsburgh, PA, USA.
Wadih El KhouryCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0000-0001-5635-9643
Rong XieCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.
Yassmin Al AarajCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0000-0002-3252-8606
Jingsi ZhaoCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.
Neha HafeezCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0000-0002-4820-0795
Rashmi J RaoCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.
Siyi JiangCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.
Vinny NegiCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.
Anna KirillovaCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.
Dror PerkCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0000-0002-5900-057X
Annie M WatsonCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0009-0002-5340-9494
Claudette M St CroixCenter for Biologic Imaging, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0000-0003-0794-4939
Donna B StolzCenter for Biologic Imaging, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0000-0002-1833-7010
Ji Young LeeLaufer Center for Physical and Quantitative Biology, Stony Brook University, Stony Brook, NY, USA.ORCID 0000-0002-1491-6963
Mary Hongying ChengLaufer Center for Physical and Quantitative Biology, Stony Brook University, Stony Brook, NY, USA.ORCID 0000-0001-5833-8221
Manling ZhangCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0000-0003-2689-1465
Samuel DetmerDepartment of Chemistry, Massachusetts Institute of Technology, Boston, MA, USA.ORCID 0009-0007-5517-2642
Edward GuzmanInstitute of Medical Engineering and Science, Massachusetts Institute of Technology, Boston, MA, USA.
Rajith S MananDepartment of Chemical Engineering, Massachusetts Institute of Technology, Boston, MA, USA.ORCID 0000-0001-7081-0138
Rajan SaggarDepartment of Medicine, David Geffen School of Medicine, University of California, Los Angeles (UCLA), Los Angeles, CA, USA.
Kathleen J HaleyDivision of Pulmonary and Critical Care Medicine, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.ORCID 0000-0002-3591-2291
Aaron B WaxmanDivision of Pulmonary and Critical Care Medicine, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.ORCID 0000-0001-7797-0361
Satoshi OkawaCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0000-0003-3916-3109
Tae-Hwi Schwantes-AnDepartment of Medical and Molecular Genetics, Indiana University, Indianapolis, IN, USA.ORCID 0000-0001-6387-0095
Michael W PauciuloDivision of Human Genetics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, USA.ORCID 0000-0002-2141-2966
Bing WangCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0000-0002-5185-3500
Amy WebbDepartment of Biomedical Informatics, Ohio State University, Columbus, OH, USA.
Caroline ChauvetUniversité Côte d'Azur, CNRS, Institut de Pharmacologie Moléculaire et Cellulaire (IPMC), Sophia-Antipolis, Valbonne, France.
Daniel G AndersonInstitute of Medical Engineering and Science, Massachusetts Institute of Technology, Boston, MA, USA.ORCID 0000-0001-5629-4798
William C NicholsDivision of Human Genetics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, USA.ORCID 0000-0003-1242-9237
Ankit A DesaiDepartment of Medicine, Indiana University, Indianapolis, IN, USA.
Robert LafyatisDivision of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0000-0002-9398-5034
S Mehdi NouraieDivision of Pulmonary, Allergy, and Critical Care Medicine, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0000-0001-7465-0581
Haodi WuCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0000-0002-7900-3073
Jeffrey G McDonaldCenter for Human Nutrition, University of Texas Southwestern Medical Center, Dallas, TX, USA.ORCID 0000-0003-1570-4142
Susan ChengDepartment of Cardiology, Smidt Heart Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA.ORCID 0000-0002-4977-036X
Ivet BaharLaufer Center for Physical and Quantitative Biology, Stony Brook University, Stony Brook, NY, USA.ORCID 0000-0001-9959-4176
Thomas BerteroUniversité Côte d'Azur, CNRS, Institut de Pharmacologie Moléculaire et Cellulaire (IPMC), Sophia-Antipolis, Valbonne, France.ORCID 0000-0002-4801-9902
Raymond L BenzaDivision of Cardiology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.ORCID 0000-0001-9627-6236
Mohit JainDepartment of Medicine, University of California, San Diego, La Jolla, CA, USA.
Stephen Y ChanCenter for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.ORCID 0000-0002-9520-7527
Funding
UT Southwestern Center for Translational MedicineUL1TR003163 · NCATS · UT SOUTHWESTERN MEDICAL CENTER · PI TOTO, ROBERT DANIEL · 2021 to 2025
$39.3MTissue Culture & Antibody Production CoreP01HL160487 · NHLBI · UT SOUTHWESTERN MEDICAL CENTER · PI Helen Haskell Hobbs · 2022 to 2026
$14.9MNational Biological Sample and Data Repository for PAHR24HL105333 · NHLBI · CINCINNATI CHILDRENS HOSP MED CTR · PI NICHOLS, WILLIAM C · 2012 to 2016
$10.1MSystems Biology CoreP50AR080612 · NIAMS · UNIVERSITY OF PITTSBURGH AT PITTSBURGH · PI Stephen Y Chan · 2022 to 2026
$9.5MUT Southwestern NORCP30DK127984 · NIDDK · UT SOUTHWESTERN MEDICAL CENTER · PI Jeffrey M Zigman · 2022 to 2026
$7.4MDefining the Complex Biology of the miR-130/301 Family in Pulmonary HypertensionR01HL124021 · NHLBI · UNIVERSITY OF PITTSBURGH AT PITTSBURGH · PI CHAN, STEPHEN Y · 2014 to 2025
$4.9MTraining Program in Imaging Sciences in Translational Cardiovascular ResearchT32HL129964 · NHLBI · UNIVERSITY OF PITTSBURGH AT PITTSBURGH · PI Flordeliza S Villanueva · 2016 to 2026
$4.7MIron-Sulfur Deficiency as a Critical Pathogenic Cause of Pulmonary HypertensionR01HL122596 · NHLBI · UNIVERSITY OF PITTSBURGH AT PITTSBURGH · PI CHAN, STEPHEN Y · 2015 to 2024
$4.5MRisk stratification in pulmonary arterial hypertension: Intersection of OMICs and longitudinal phenotypes through the PAH BiobankR01HL160941 · NHLBI · CINCINNATI CHILDRENS HOSP MED CTR · PI DESAI, ANKIT A, NICHOLS, WILLIAM C · 2022 to 2025
$3.2MGenetic and hypoxic control of a lncRNA axis orchestrates endothelial reprogramming in pulmonary hypertensionR01HL151228 · NHLBI · UNIVERSITY OF PITTSBURGH AT PITTSBURGH · PI Stephen Y Chan · 2023 to 2026
$2.8MToward a Deeper Understanding of Allostery and Allotargeting by Computational ApproachesR01GM139297 · NIGMS · UNIVERSITY OF PITTSBURGH AT PITTSBURGH · PI Ivet Bahar · 2021 to 2026
$2.8MPharmacogenomics in Pulmonary Arterial HypertensionR01HL078946 · NHLBI · UNIVERSITY OF ALABAMA AT BIRMINGHAM · PI BENZA, RAYMOND LOUIS · 2005 to 2008
$1.8MNCATS NIH HHS UL1 TR003163NHLBI NIH HHS F30 HL143879NHLBI NIH HHS F30 HL170649NHLBI NIH HHS K08 HL166950NHLBI NIH HHS P01 HL160487NHLBI NIH HHS R00 HL161320NHLBI NIH HHS R01 HL078946NHLBI NIH HHS R01 HL122596NHLBI NIH HHS R01 HL124021NHLBI NIH HHS R01 HL151228NHLBI NIH HHS R01 HL160941NHLBI NIH HHS R24 HL105333NHLBI NIH HHS T32 HL129964NIAMS NIH HHS P50 AR080612NIDDK NIH HHS P30 DK127984NIGMS NIH HHS R01 GM139297NIH HHS S10 OD023684NIH HHS S10 OD025041
Abstract
Vascular inflammation regulates endothelial pathophenotypes, particularly in pulmonary arterial hypertension (PAH). Dysregulated lysosomal activity and cholesterol metabolism activate pathogenic inflammation, but their relevance to PAH is unclear. Nuclear receptor coactivator 7 (
Indexed as
LysosomesNuclear Receptor CoactivatorsOxysterolsPulmonary Arterial HypertensionAnimalsBile Acids and SaltsEndothelium, VascularFemaleGenetic Predisposition to DiseaseHumansInflammationMaleMiceMice, Inbred C57BLMice, KnockoutPolymorphism, Single NucleotideBile Acids and SaltsNCOA7 protein, humanNcoa7 protein, mouseNuclear Receptor CoactivatorsOxysterols
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.