Core C: Imaging-Abou Alaiwa

核心C：成像-Abou Alaiwa

• 批准号: 10470208
• 负责人: Mahmoud Abou Alaiwa
• 金额: $ 15.53万
• 依托单位: UNIVERSITY OF IOWA
• 依托单位国家: 美国
• 财政年份: 2008
• 资助国家: 美国
• 起止时间: 2008-09-05 至 2024-06-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/10470208
• 关键词: ATP12A gene; Address; Affect; Airway Disease; Animal Model; Biliary cirrhosis; Biological Assay; Chronic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Data Analyses; Data Set; Disease; Disease Progression; Ensure; Exocrine pancreas; Family suidae; Gallbladder; Genes; Goals; High Resolution Computed Tomography; Human; Human Resources; Image; Infection; Inflammation; Intestinal Obstruction; Intestines; Iowa; Knock-out; Knowledge; Life; Liver; Lung; Maintenance; Measures; Modeling; Monitor; Morbidity - disease rate; Mucociliary Clearance; Mutation; Neonatal; Organ; Pancreas; Pathogenesis; Patients; Phenotype; Prevention; Program Research Project Grants; Pulmonary Cystic Fibrosis; Research; Resources; Respiratory Failure; Respiratory Tract Infections; Sinus; Structural defect; Sweat Glands; Technology; Time; Universities; Vas deferens structure; X-Ray Computed Tomography; airway inflammation; base; cystic fibrosis airway; detector; disease-causing mutation; imaging facilities; imaging modality; improved; lung imaging; male; mortality; novel; porcine model; programs; recurrent infection; tool

CORE C – IMAGING PROJECT SUMMARY Cystic fibrosis (CF) is a common autosomal recessive disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR). CF affects multiple organs, including lungs, pancreas, intestine, liver, sweat glands, gallbladder and the male productive tract. Airway infection and inflammation currently cause most of the morbidity and mortality. Although several therapies have improved the lives of patients, current treatments are inadequate and CF remains a lethal disease. Our knowledge about the pathogenesis and the progression of the disease, as well as the state of the neonatal lung is inadequate. These gaps in our knowledge have hindered attempts to develop better treatments and preventions for CF lung disease. A major impediment to addressing these issues has been limitations of current animal models. CF pigs replicate many of the key features of human CF disease including intestinal obstruction, exocrine pancreatic destruction, micro-gallbladder, vas deferens abnormalities, focal biliary cirrhosis, congenital airway structural abnormalities, and airway and sinus infection with time. The Imaging Core has two main objectives: (1) Provide scientific support, expertise, and resources for acquisition of image datasets and subsequent analysis; and (2) Help Project Leaders successfully accomplish the aims of their Projects. State-of-the-art image acquisition technology and analysis tools are readily available at the University of Iowa for these Projects. The Imaging Core will function seamlessly through already established interactions with the Project Leaders, Core Directors, the Translational Lung Imaging Research Program, and the Iowa Comprehensive Lung Imaging Center.

核心C -成像 项目摘要 囊性纤维化（CF）是一种常见的常染色体隐性遗传病，由编码 囊性纤维化跨膜传导调节因子（CFTR）。CF影响多个器官，包括肺， 胰腺、肠、肝、汗腺、胆囊和男性生殖道。呼吸道感染和 炎症目前引起大部分的发病率和死亡率。虽然有几种疗法已经有所改善 然而，由于CF严重威胁患者的生命，目前的治疗是不充分的，并且CF仍然是致命的疾病。我们的知识 关于发病机制和疾病的进展，以及新生儿肺的状态， 不足我们知识上的这些差距阻碍了开发更好的治疗方法的尝试， 预防CF肺病。解决这些问题的一个主要障碍是 目前的动物模型。CF猪复制了人类CF疾病的许多关键特征，包括肠 梗阻，胰腺外分泌破坏，微胆囊，输精管异常，局灶性胆道 肝硬化、先天性气道结构异常和气道和鼻窦感染。成像 核心有两个主要目标：（1）提供科学支持，专业知识和资源，以获取图像 数据集和后续分析;（2）帮助项目负责人成功实现其目标 项目最先进的图像采集技术和分析工具可随时在 爱荷华州大学为这些项目。成像核心将通过已经建立的 与项目负责人、核心负责人、转化肺成像研究项目的互动，以及 爱荷华州综合肺部成像中心。