DYSREGULATED NFKB PATHWAY SIGNALING IN MYELOPROLIFERATIVE NEOPLASMS

7.项目总结/摘要 骨髓增生性肿瘤（MPN）是一种慢性血液疾病，可导致严重的症状， 早死最近出现了有助于改善症状的新治疗方法，但事实并非如此 可靠地减缓或停止疾病进展。我们寻求更好地了解是什么驱动了疾病的发展， 因此，我们可以为患有这些疾病的患者开发更好的治疗方法。 我们的初步数据表明，一种称为NFkB通路的信号通路在细胞内被异常激活。 MPN。我们推测，这一途径有助于MPN的发展和进展。因此，我们认为， 我们已经提出了一个小鼠和人类研究的组合，以确定如何NF κ B途径 有助于MPN的发病机制，并评估抑制NF κ B信号传导是否可能具有潜在的 对MPN患者的治疗益处。

7. Project Summary/Abstract Myeloproliferative neoplasms (MPNs) are chronic blood disorders that that can cause severe symptoms and early death. New treatments have become available recently that help ameliorate symptoms, but they do not reliably slow or halt disease progression. We seek to better understand what drives disease development and progression in MPNs, so that we can develop better therapies for patients with these diseases. Our preliminary data indicates that a signaling pathway called the NFkB pathway is abnormally activated in MPNs. We hypothesize that this pathway contributes to the development and progression of MPNs. Therefore, we have proposed a combination of mouse and human studies to determine how the NFkB pathway contributes to MPN pathogenesis, and to evaluate whether inhibition of NFkB signaling may have potential therapeutic benefits for MPN patients.