| Pulmonary vascular disease in mice xenografted with human BM progenitors from patients with pulmonary arterial hypertension. | |
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MedLine Citation:
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PMID: 22745307 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Hematopoietic myeloid progenitors released into the circulation are able to promote vascular remodeling through endothelium activation and injury. Endothelial injury is central to the development of pulmonary arterial hypertension (PAH), a proliferative vasculopathy of the pulmonary circulation, but the origin of vascular injury is unknown. In the present study, mice transplanted with BM-derived CD133(+) progenitor cells from patients with PAH, but not from healthy controls, exhibited morbidity and/or death due to features of PAH: in situ thrombi and endothelial injury, angioproliferative remodeling, and right ventricular hypertrophy and failure. Myeloid progenitors from patients with heritable and/or idiopathic PAH all produced disease in xenografted mice. Analyses of hematopoietic transcription factors and colony formation revealed underlying abnormalities of progenitors that skewed differentiation toward the myeloid-erythroid lineage. The results of the present study suggest a causal role for hematopoietic stem cell abnormalities in vascular injury, right ventricular hypertrophy, and morbidity associated with PAH. |
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Authors:
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Kewal Asosingh; Samar Farha; Alan Lichtin; Brian Graham; Deepa George; Micheala Aldred; Stanley L Hazen; James Loyd; Rubin Tuder; Serpil C Erzurum |
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Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't Date: 2012-06-28 |
Journal Detail:
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Title: Blood Volume: 120 ISSN: 1528-0020 ISO Abbreviation: Blood Publication Date: 2012 Aug |
Date Detail:
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Created Date: 2012-08-10 Completed Date: 2012-10-25 Revised Date: 2013-05-08 |
Medline Journal Info:
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Nlm Unique ID: 7603509 Medline TA: Blood Country: United States |
Other Details:
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Languages: eng Pagination: 1218-27 Citation Subset: AIM; IM |
Affiliation:
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Pathobiology, Lerner Research Institute and Respiratory Institute, Cleveland Clinic, Cleveland, OH 44195, USA. asosink@ccf.org |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Animals Bone Marrow Cells / pathology Bone Marrow Transplantation / adverse effects*, methods Cells, Cultured Female Humans Hypertension, Pulmonary / pathology* Lung Diseases / etiology* Mice Mice, Inbred NOD Mice, SCID Pulmonary Artery / pathology Stem Cell Transplantation / adverse effects*, methods Transplantation, Heterologous / adverse effects Vascular Diseases / etiology* |
| Grant Support | |
ID/Acronym/Agency:
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HL60917/HL/NHLBI NIH HHS; K08 HL105536/HL/NHLBI NIH HHS; M01 RR018390/RR/NCRR NIH HHS; P01 HL103453/HL/NHLBI NIH HHS; P01HL076491/HL/NHLBI NIH HHS; P30 CA43703/CA/NCI NIH HHS; R01 HL098199/HL/NHLBI NIH HHS; UL1 TR000439/TR/NCATS NIH HHS |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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