Document Detail


Pulmonary vascular disease in mice xenografted with human BM progenitors from patients with pulmonary arterial hypertension.
MedLine Citation:
PMID:  22745307     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
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:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2012-06-28
Journal Detail:
Title:  Blood     Volume:  120     ISSN:  1528-0020     ISO Abbreviation:  Blood     Publication Date:  2012 Aug 
Date Detail:
Created Date:  2012-08-10     Completed Date:  2012-10-25     Revised Date:  2013-08-13    
Medline Journal Info:
Nlm Unique ID:  7603509     Medline TA:  Blood     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1218-27     Citation Subset:  AIM; IM    
Affiliation:
Pathobiology, Lerner Research Institute and Respiratory Institute, Cleveland Clinic, Cleveland, OH 44195, USA. asosink@ccf.org
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MeSH Terms
Descriptor/Qualifier:
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:
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; R37 HL060917/HL/NHLBI NIH HHS; UL1 TR000439/TR/NCATS NIH HHS
Comments/Corrections

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