NASAL ELECTRIC POTENTIAL DIFFERENCE IN NORMAL AND CYSTIC FIBROSIS PATIENTS

正常和囊性纤维化患者的鼻电位差异

• 批准号: 7604932
• 负责人: Michael John Welsh
• 金额: $ 0.16万
• 依托单位: UNIVERSITY OF IOWA
• 依托单位国家: 美国
• 财政年份: 2007
• 资助国家: 美国
• 起止时间: 2007-03-01 至 2007-09-16
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7604932
• 关键词: Affect; Amiloride; Bronchiectasis; Caucasians; Caucasoid Race; Chloride Channels; Chloride Ion; Chlorides; Computer Retrieval of Information on Scientific Projects Database; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Data; Defect; Diagnostic; Disease; Electrolytes; End Point; Epithelium; Funding; Gene Transfer; Genes; Grant; Hereditary Disease; Institution; Intervention; Measurement; Measures; Mutation; Nasal Epithelium; Nose; Pathogenesis; Perfusion; Protocols documentation; Pulmonary Cystic Fibrosis; Research; Research Personnel; Resources; Sodium; Source; United States National Institutes of Health; airway epithelium; apical membrane; cystic fibrosis patients; in vivo; response; voltage

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 of Caucasians. CF is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) which forms a chloride channel in the apical membrane of affected epithelia. As a result of the defective function of the CFTR, affected epithelia have abnormal transepithelial electrolyte transport. In airway epithelia, including the nasal epithelium, the cystic fibrosis defect is measured as an abnormally increased voltage and an abnormal response to agents that modulate chloride transport. The lack of chloride transport is a diagnostic criteria for CF. In addition, an increase in basal Vt and the change in Vt after perfusion with amiloride have been implicated by others in the pathogenesis of CF. However, previous data suggest this may not be of significance for the pathogenesis of the disease. Thus, these investigators have developed three hypotheses: a) nasal Vt measurement can be used as an end point for evaluating CFTR function and CFTR gene transfer to airway epithelia in vivo; b) people wihout CF but with bronchiectasis will have an abnormal sodium or chloride transport as measured by the nasal Vt protocol; and c) interventions that increase the VtAmil will not result in cystic fibrosis lung disease.

该子项目是利用该技术的众多研究子项目之一 资源由 NIH/NCRR 资助的中心拨款提供。子项目和 研究者 (PI) 可能已从 NIH 的另一个来源获得主要资金， 因此可以在其他 CRISP 条目中表示。列出的机构是 对于中心来说，它不一定是研究者的机构。 囊性纤维化（CF）是白种人最常见的致命遗传病。 CF 是由编码囊性纤维化跨膜电导调节因子 (CFTR) 的基因突变引起的，CFTR 在受影响的上皮细胞的顶膜中形成氯离子通道。 由于 CFTR 功能缺陷，受影响的上皮细胞具有异常的跨上皮电解质转运。 在气道上皮（包括鼻上皮）中，囊性纤维化缺陷被测量为电压异常升高以及对调节氯离子转运的药物的异常反应。 缺乏氯离子转运是 CF 的诊断标准。 此外，基础 Vt 的增加和阿米洛利灌注后 Vt 的变化也被其他人认为与 CF 的发病机制有关。 然而，之前的数据表明这对于该疾病的发病机制可能并不重要。 因此，这些研究人员提出了三个假设： a) 鼻 Vt 测量可用作评估 CFTR 功能和体内 CFTR 基因转移至气道上皮的终点； b) 没有 CF 但患有支气管扩张的人会出现通过鼻 Vt 方案测量的钠或氯转运异常； c) 增加 VtAmil 的干预措施不会导致囊性纤维化肺病。