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

点击翻译按钮获取中文摘要

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.