THE ROLE OF PROCOLLAGEN 1 AND TGF-BETA 1 IN CYSTICFIBROTIC PULMONARY DISEASE

本子项目是利用由NIH/NCRR资助的中心赠款提供的资源的众多研究子项目之一。子项目和研究者（PI）可能已经从另一个NIH来源获得了主要资金，因此可以在其他CRISP条目中表示。列出的机构是中心的，不一定是研究者的机构。囊性纤维化（CF）是白种人中最常见的致死性遗传病。CF患者肺组织病理学和超微结构研究显示粘膜下炎症伴中性粒细胞、淋巴细胞和浆细胞，粘膜下基质破坏和修复。胶原I和胶原III都存在于上皮下纤维化层。TGF-b多态性与CF的预后有关，据报道TGF-b1存在于CF气道中。TGF-b是调节炎症细胞功能和细胞外基质生成的多肽生长因子家族的一员。在本提案中，我们将确定CF患者的痰是否可以上调前胶原I的产生，这是由于TGF-b1。此外，我们将确定前胶原蛋白1的上调程度或TGF-b1水平是否与临床结果相关。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Cystic fibrosis (CF) is the most common lethal genetic disease among Caucasians. Histopathologic and ultrastructural studies of lungs from CF patients have revealed submucosal inflammation with neutrophils, lymphocytes and plasma cells as well submucosal matrix destruction and repair. Both collagen I and collagen III have are present in the subepithelial fibrosis layer. TGF-b polymorphisms have been linked to outcome in CF and TGF-b1 has been reported to be present in the CF airway. TGF-b is a member of a family of polypeptide growth factors which modulate inflammatory cell function as well as extracellular matrix production. In this proposal we will determine if sputum from CF patients can upregulate procollagen I production and this is due to TGF-b1. In addition we will determine if the degree of procollagen 1 upregulation or TGF-b1 levels are associated with clinical outcome.