Molecular mechanisms of reduced expression of the angiostatic chemokine IP-10 in idiopathic pulmonary fibrosis

特发性肺纤维化（IPF）是一种进行性和致死性肺部疾病，其特征是不可逆的肺部瘢痕形成，目前尚无有效的治疗方法。过度的成纤维细胞（一种细胞类型）生长和疤痕形成是该疾病最重要的特征，需要新的血管形成来适应营养需求。在正常的肺中，新血管的形成被一种叫做IP-10的抑制剂蛋白所抑制。然而，在IPF肺中，IP-10的产生减少，导致新血管形成和无对抗的成纤维细胞生长和疤痕形成。IPF肺中IP-10表达异常的原因尚不清楚，这是一个重要的研究领域。我们认为IPF中IP-10表达的关键过程存在缺陷。在本文提出的研究中，我们将比较IPF患者的成纤维细胞与正常细胞，以研究调节ip10表达的分子机制，并确定IPF中的缺陷过程。这将有助于我们更好地了解这种疾病，并可能导致恢复IP-10表达的方法的发展，作为一种新的治疗IPF的方法。

Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal lung disorder characterised by irreversible scarring of the lung and has no effective therapy. Excessive fibroblast (a cell type) growth and scar formation are the most important features of the disease and require new blood vessel formation to accommodate the nutritional need. In normal lung the new blood vessel formation is inhibited by an inhibitor protein called IP-10. However, in IPF lung, IP-10 production is reduced, leading to new blood vessel formation and unopposed fibroblast growth and scar formation. Why IP-10 expression is faulty in IPF lung is unclear and is an important area to study. We believe that key processes in IP-10 expression are defective in IPF. In the studies proposed here we will compare fibroblasts from IPF patients with normal cells to examine the molecular mechanisms regulating the IP-10 expression and identify the defective processes in IPF. This will help us to understand the disease better and may lead to the development of approaches to restore IP-10 expression as a new treatment for IPF.