{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T19:46:16Z","timestamp":1790279176699,"version":"4.1.0"},"reference-count":29,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2023,6,7]],"date-time":"2023-06-07T00:00:00Z","timestamp":1686096000000},"content-version":"vor","delay-in-days":67,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["SFB 1118"],"award-info":[{"award-number":["SFB 1118"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["FerrOs \u2010 FOR5146"],"award-info":[{"award-number":["FerrOs \u2010 FOR5146"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["SPP 2306"],"award-info":[{"award-number":["SPP 2306"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pulm. circ."],"published-print":{"date-parts":[[2023,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Iron deficiency is common in idiopathic and heritable pulmonary arterial hypertension patients (I\/HPAH). A previous report suggested a dysregulation of the iron hormone hepcidin, which is controlled by BMP\/SMAD signaling involving the bone morphogenetic protein receptor 2 (BMPR\u2010II). Pathogenic variants in the <jats:italic>BMPR2<\/jats:italic> gene are the most common cause of HPAH. Their effect on patients'\u00a0hepcidin levels has not been investigated. The aim of this study was to assess whether iron metabolism and regulation of the iron regulatory hormone hepcidin was disturbed in I\/HPAH patients with and without a pathogenic variant in the gene <jats:italic>BMPR2<\/jats:italic> compared to healthy controls. In this explorative, cross\u2010sectional study hepcidin serum levels were quantified by enzyme\u2010linked immunosorbent assay. We measured iron status, inflammatory parameters and hepcidin modifying proteins such as IL6, erythropoietin, and BMP2, BMP6 in addition to BMPR\u2010II protein and mRNA levels. Clinical routine parameters were correlated with hepcidin levels. In total 109 I\/HPAH patients and controls, separated into three groups, 23 <jats:italic>BMPR2<\/jats:italic> variant\u2010carriers, 56 <jats:italic>BMPR2<\/jats:italic> noncarriers and 30 healthy controls were enrolled. Of these, 84% had iron deficiency requiring iron supplementation. Hepcidin levels were not different between groups and corresponded to the degree of iron deficiency. The levels of IL6, erythropoietin, BMP2, or BMP6 showed no correlation with hepcidin expression. Hence, iron homeostasis and hepcidin regulation was largely independent from these parameters. I\/HPAH patients had a physiologically normal iron regulation and no false elevation of hepcidin levels. Iron deficiency was prevalent albeit independent of pathogenic variants in the <jats:italic>BMPR2<\/jats:italic> gene.<\/jats:p>","DOI":"10.1002\/pul2.12242","type":"journal-article","created":{"date-parts":[[2023,6,8]],"date-time":"2023-06-08T04:34:54Z","timestamp":1686198894000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Is iron deficiency caused by <i>BMPR2<\/i> mutations or dysfunction in pulmonary arterial hypertension patients?"],"prefix":"10.1002","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0009-0005-7117-1346","authenticated-orcid":false,"given":"Vivienne","family":"Theobald","sequence":"first","affiliation":[{"name":"Center for Pulmonary Hypertension Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital Heidelberg Germany"},{"name":"Translational Lung Research Center Heidelberg (TLRC) German Center for Lung Research (DZL)  Heidelberg 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Leipzig Leipzig Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Halank","sequence":"additional","affiliation":[{"name":"Medical Clinic I University Hospital of Dresden  Dresden Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc A.","family":"Schneider","sequence":"additional","affiliation":[{"name":"Translational Lung Research Center Heidelberg (TLRC) German Center for Lung Research (DZL)  Heidelberg Germany"},{"name":"Translational Research Unit Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7368-8297","authenticated-orcid":false,"given":"Sarah","family":"Richtmann","sequence":"additional","affiliation":[{"name":"Translational Lung Research Center Heidelberg (TLRC) German Center for Lung Research (DZL)  Heidelberg Germany"},{"name":"Translational Research Unit Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Kazdal","sequence":"additional","affiliation":[{"name":"Translational Lung Research Center Heidelberg (TLRC) German Center for Lung Research (DZL)  Heidelberg Germany"},{"name":"Institute of Pathology Heidelberg University Hospital Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katrin","family":"Hinderhofer","sequence":"additional","affiliation":[{"name":"Laboratory for Molecular Diagnostics, Institute of Human Genetics Heidelberg University Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Panagiota","family":"Xanthouli","sequence":"additional","affiliation":[{"name":"Center for Pulmonary Hypertension Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital Heidelberg Germany"},{"name":"Translational Lung Research Center Heidelberg (TLRC) German Center for Lung Research (DZL)  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin","family":"Egenlauf","sequence":"additional","affiliation":[{"name":"Center for Pulmonary Hypertension Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital Heidelberg Germany"},{"name":"Translational Lung Research Center Heidelberg (TLRC) German Center for Lung Research (DZL)  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satenik","family":"Harutyunova","sequence":"additional","affiliation":[{"name":"Center for Pulmonary Hypertension Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital Heidelberg Germany"},{"name":"Translational Lung Research Center Heidelberg (TLRC) German Center for Lung Research (DZL)  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marius M.","family":"Hoeper","sequence":"additional","affiliation":[{"name":"Department of Pneumology, Hannover Medical School, Biomedical Research in End\u2010stage and Obstructive Lung Disease Hannover (BREATH) German Center for Lung Research (DZL) Hannover Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Danny","family":"Jonigk","sequence":"additional","affiliation":[{"name":"Hannover Medical School, Institute for Pathology, German Center for Lung Research (DZL) Biomedical Research in End\u2010stage and Obstructive Lung Disease Hannover (BREATH) Hannover Germany"},{"name":"Institute of Pathology RWTH Aachen University Hospital  Aachen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Sparla","sequence":"additional","affiliation":[{"name":"Translational Lung Research Center Heidelberg (TLRC) German Center for Lung Research (DZL)  Heidelberg Germany"},{"name":"Centre for Translational Biomedical Iron Research, Hematology, Immunology and Pulmonology University Hospital Heidelberg Heidelberg 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