Missense Mutations of the <i>BMPR1B</i> (<i>ALK6</i>) Gene in Childhood Idiopathic Pulmonary Arterial Hypertension
نویسندگان
چکیده
منابع مشابه
Mutations of NOTCH3 in childhood pulmonary arterial hypertension
Mutations of BMPR2 and other TGF-β superfamily genes have been reported in pulmonary arterial hypertension (PAH). However, 60-90% of idiopathic PAH cases have no mutations in these genes. Recently, the expression of NOTCH3 was shown to be increased in the pulmonary artery smooth muscle cells of PAH patients. We sought to investigate NOTCH3 and its target genes in PAH patients and clarify the ro...
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cytometry analysis of BAL cells. Table 1 integrates information given in our published paper, clearly demonstrating that the number of BAL neutrophils was fair in our case series. This unfortunately prevented a definitive evaluation of whether polymorph nucleates represent a source of IL-17. Nonetheless, as shown in figure 1, in selected cases with a significant number of BAL neutrophils (two s...
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cytometry analysis of BAL cells. Table 1 integrates information given in our published paper, clearly demonstrating that the number of BAL neutrophils was fair in our case series. This unfortunately prevented a definitive evaluation of whether polymorph nucleates represent a source of IL-17. Nonetheless, as shown in figure 1, in selected cases with a significant number of BAL neutrophils (two s...
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The purpose of this investigation was to assess the effect of captopril on both systemic (P.a) and pulmonary arterial pressures (PPA) in patients with high-altitude pulmonary hypertension (HAPH). Seventeen patients (mean age 44±6.8 years) with HAPH and mild to moderate systemic arterial hypertension were included in the study. All patients underwent right heart catheterization with measurem...
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AIMS To determine the diagnostic utility of circulating angiopoietin-1 (Ang-1) and its antagonist angiopoietin-2 (Ang-2) as potential biomarkers of disease severity or response to treatment in idiopathic pulmonary arterial hypertension (IPAH). Imbalances in angiogenic factors including vascular endothelial cell growth factor (VEGF) and the angiopoetin-Tie2 receptor system have been implicated i...
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ژورنال
عنوان ژورنال: Circulation Journal
سال: 2012
ISSN: 1346-9843,1347-4820
DOI: 10.1253/circj.cj-11-1281