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Lysosomal dysfunction and inflammatory sterol metabolism in pulmonary arterial hypertension.

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Title: Lysosomal dysfunction and inflammatory sterol metabolism in pulmonary arterial hypertension.
Authors: Harvey LD; Medical Scientist Training Program, University of Pittsburgh, Pittsburgh, PA, USA.; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Alotaibi M; Division of Pulmonary and Critical Care Medicine, University of California San Diego, La Jolla, CA, USA.; Department of Medicine, University of California, San Diego, La Jolla, CA, USA.; Department of Pharmacology, University of California, San Diego, La Jolla, CA, USA., Tai YY; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Tang Y; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Kim HJ; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Kelly NJ; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.; Division of Cardiology, Veterans Affairs Pittsburgh Healthcare System, Pittsburgh, PA, USA., Sun W; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Woodcock CC; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Arshad S; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Culley MK; Medical Scientist Training Program, University of Pittsburgh, Pittsburgh, PA, USA.; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., El Khoury W; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Xie R; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Al Aaraj Y; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Zhao J; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Hafeez N; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Rao RJ; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Jiang S; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Negi V; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Kirillova A; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Perk D; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Watson AM; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., St Croix CM; Center for Biologic Imaging, University of Pittsburgh, Pittsburgh, PA, USA., Stolz DB; Center for Biologic Imaging, University of Pittsburgh, Pittsburgh, PA, USA., Lee JY; Laufer Center for Physical and Quantitative Biology, Stony Brook University, Stony Brook, NY, USA.; Department of Biochemistry and Cell Biology, Stony Brook University, Stony Brook, NY, USA., Cheng MH; Laufer Center for Physical and Quantitative Biology, Stony Brook University, Stony Brook, NY, USA.; Department of Biochemistry and Cell Biology, Stony Brook University, Stony Brook, NY, USA., Zhang M; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.; Division of Cardiology, Veterans Affairs Pittsburgh Healthcare System, Pittsburgh, PA, USA., Detmer S; Department of Chemistry, Massachusetts Institute of Technology, Boston, MA, USA., Guzman E; Institute of Medical Engineering and Science, Massachusetts Institute of Technology, Boston, MA, USA.; School of Engineering and Applied Sciences, Harvard University, Cambridge, MA, USA., Manan RS; Department of Chemical Engineering, Massachusetts Institute of Technology, Boston, MA, USA., Saggar R; Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles (UCLA), Los Angeles, CA, USA.; Department of Pathology, David Geffen School of Medicine, UCLA, Los Angeles, CA, USA., Haley KJ; Division of Pulmonary and Critical Care Medicine, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA., Waxman AB; Division of Pulmonary and Critical Care Medicine, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA., Okawa S; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.; Department of Computational and Systems Biology, University of Pittsburgh, Pittsburgh, PA, USA.; McGowan Institute for Regenerative Medicine, University of Pittsburgh, Pittsburgh, PA, USA., Schwantes-An TH; Department of Medical and Molecular Genetics, Indiana University, Indianapolis, IN, USA.; Department of Medicine, Indiana University, Indianapolis, IN, USA., Pauciulo MW; Division of Human Genetics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, USA., Wang B; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Webb A; Department of Biomedical Informatics, Ohio State University, Columbus, OH, USA., Chauvet C; Université Côte d'Azur, CNRS, Institut de Pharmacologie Moléculaire et Cellulaire (IPMC), Sophia-Antipolis, Valbonne, France., Anderson DG; Institute of Medical Engineering and Science, Massachusetts Institute of Technology, Boston, MA, USA., Nichols WC; Division of Human Genetics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, USA., Desai AA; Department of Medicine, Indiana University, Indianapolis, IN, USA., Lafyatis R; Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, USA., Nouraie SM; Division of Pulmonary, Allergy, and Critical Care Medicine, University of Pittsburgh, Pittsburgh, PA, USA., Wu H; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., McDonald JG; Center for Human Nutrition, University of Texas Southwestern Medical Center, Dallas, TX, USA.; Department of Molecular Genetics, University of Texas Southwestern Medical Center, Dallas, TX, USA., Cheng S; Department of Cardiology, Smidt Heart Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA., Bahar I; Laufer Center for Physical and Quantitative Biology, Stony Brook University, Stony Brook, NY, USA.; Department of Biochemistry and Cell Biology, Stony Brook University, Stony Brook, NY, USA., Bertero T; Université Côte d'Azur, CNRS, Institut de Pharmacologie Moléculaire et Cellulaire (IPMC), Sophia-Antipolis, Valbonne, France., Benza RL; Division of Cardiology, Icahn School of Medicine at Mount Sinai, New York, NY, USA., Jain M; Department of Medicine, University of California, San Diego, La Jolla, CA, USA.; Department of Pharmacology, University of California, San Diego, La Jolla, CA, USA., Chan SY; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
Source: Science (New York, N.Y.) [Science] 2025 Jan 24; Vol. 387 (6732), pp. eadn7277. Date of Electronic Publication: 2025 Jan 24.
Publication Type: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.; Research Support, N.I.H., Extramural
Journal Info: Publisher: American Association for the Advancement of Science Country of Publication: United States NLM ID: 0404511 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1095-9203 (Electronic) Linking ISSN: 00368075 NLM ISO Abbreviation: Science Subsets: MEDLINE
Database: MEDLINE Ultimate
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  Data: Lysosomal dysfunction and inflammatory sterol metabolism in pulmonary arterial hypertension.
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Medical Scientist Training Program, University of Pittsburgh, Pittsburgh, PA, USA.; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.<br /><searchLink fieldCode="AU" term="%22El+Khoury+W%22">El Khoury W</searchLink>; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.<br /><searchLink fieldCode="AU" term="%22Xie+R%22">Xie R</searchLink>; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.<br /><searchLink fieldCode="AU" term="%22Al+Aaraj+Y%22">Al Aaraj Y</searchLink>; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.<br /><searchLink fieldCode="AU" term="%22Zhao+J%22">Zhao J</searchLink>; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.<br /><searchLink fieldCode="AU" term="%22Hafeez+N%22">Hafeez N</searchLink>; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.<br /><searchLink fieldCode="AU" term="%22Rao+RJ%22">Rao RJ</searchLink>; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; 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Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.<br /><searchLink fieldCode="AU" term="%22Watson+AM%22">Watson AM</searchLink>; Center for Pulmonary Vascular Biology and Medicine, Pittsburgh, Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA, USA.; Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.<br /><searchLink fieldCode="AU" term="%22St+Croix+CM%22">St Croix CM</searchLink>; Center for Biologic Imaging, University of Pittsburgh, Pittsburgh, PA, USA.<br /><searchLink fieldCode="AU" term="%22Stolz+DB%22">Stolz DB</searchLink>; Center for Biologic Imaging, University of Pittsburgh, Pittsburgh, PA, USA.<br /><searchLink fieldCode="AU" term="%22Lee+JY%22">Lee JY</searchLink>; Laufer Center for Physical and Quantitative Biology, Stony Brook University, Stony Brook, NY, USA.; 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      – PersonEntity:
          Name:
            NameFull: Cheng MH
      – PersonEntity:
          Name:
            NameFull: Zhang M
      – PersonEntity:
          Name:
            NameFull: Detmer S
      – PersonEntity:
          Name:
            NameFull: Guzman E
      – PersonEntity:
          Name:
            NameFull: Manan RS
      – PersonEntity:
          Name:
            NameFull: Saggar R
      – PersonEntity:
          Name:
            NameFull: Haley KJ
      – PersonEntity:
          Name:
            NameFull: Waxman AB
      – PersonEntity:
          Name:
            NameFull: Okawa S
      – PersonEntity:
          Name:
            NameFull: Schwantes-An TH
      – PersonEntity:
          Name:
            NameFull: Pauciulo MW
      – PersonEntity:
          Name:
            NameFull: Wang B
      – PersonEntity:
          Name:
            NameFull: Webb A
      – PersonEntity:
          Name:
            NameFull: Chauvet C
      – PersonEntity:
          Name:
            NameFull: Anderson DG
      – PersonEntity:
          Name:
            NameFull: Nichols WC
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          Name:
            NameFull: Desai AA
      – PersonEntity:
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            NameFull: Lafyatis R
      – PersonEntity:
          Name:
            NameFull: Nouraie SM
      – PersonEntity:
          Name:
            NameFull: Wu H
      – PersonEntity:
          Name:
            NameFull: McDonald JG
      – PersonEntity:
          Name:
            NameFull: Cheng S
      – PersonEntity:
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            NameFull: Bahar I
      – PersonEntity:
          Name:
            NameFull: Bertero T
      – PersonEntity:
          Name:
            NameFull: Benza RL
      – PersonEntity:
          Name:
            NameFull: Jain M
      – PersonEntity:
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            NameFull: Chan SY
    IsPartOfRelationships:
      – BibEntity:
          Dates:
            – D: 24
              M: 01
              Text: 2025 Jan 24
              Type: published
              Y: 2025
          Identifiers:
            – Type: issn-electronic
              Value: 1095-9203
          Numbering:
            – Type: volume
              Value: 387
            – Type: issue
              Value: 6732
          Titles:
            – TitleFull: Science (New York, N.Y.)
              Type: main
ResultId 1