期刊
BLOOD
卷 120, 期 6, 页码 1218-1227出版社
AMER SOC HEMATOLOGY
DOI: 10.1182/blood-2012-03-419275
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资金
- National Institutes of Health [HL60917, P01 HL103453, P01HL076491, M01 RR018390]
- American Thoracic Society/Pulmonary Association Research [PH-07-003]
- Hematopoietic Stem Cell Core Facility of the Case Comprehensive Cancer Center [P30 CA43703]
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. (Blood. 2012;120(6):1218-1227)
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