TRANSEPITHELIAL POTENTIAL DIFFERENCE IN PTS W/ VARIANTS OF CYSTIC FIBROSIS (CF)

囊性纤维化 (CF) 变体 PTS 的跨上皮电位差

• 批准号: 7604525
• 负责人: Pamela L. Zeitlin
• 金额: $ 0.03万
• 依托单位: JOHNS HOPKINS UNIVERSITY
• 依托单位国家: 美国
• 财政年份: 2006
• 资助国家: 美国
• 起止时间: 2006-12-01 至 2007-09-16
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7604525
• 关键词: Amiloride; Chloride Ion; Chlorides; Class; Computer Retrieval of Information on Scientific Projects Database; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Funding; Genotype; Grant; Institution; Isoproterenol; Measurement; Mutation; Nose; Patients; Physicians; Research; Research Personnel; Resources; Role; Source; Surrogate Markers; United States National Institutes of Health; Variant; base; neodymium pyrocatechin disulfonate; novel diagnostics; response; tool

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. Nasal Potenial Difference Measurements are a new diagnostic tool in CF. The hypothesis of this study is that Class IV or V mutations in CFTR will be associated with chloride transport in the NPD. The specific aims are 1) to correlate the magnitude of the change in NPD response to low chloride/isoproterenol/amiloride with CFTR genotype; 2) to expand the understanding of the relationship between potential surrogate markers and genotype in CF; and to substantiate the role of NPD measurement as a valuable research tool. Patients are referred by physicians who find some but not all features of CF, or on the basis of CFTR genotype.

该子项目是利用 NIH/NCRR 资助的中心拨款提供的资源的众多研究子项目之一。子项目和研究者 (PI) 可能已从另一个 NIH 来源获得主要资金，因此可以在其他 CRISP 条目中得到体现。列出的机构是中心的机构，不一定是研究者的机构。鼻电位差测量是 CF 的一种新诊断工具。本研究的假设是 CFTR 中的 IV 类或 V 类突变将与 NPD 中的氯离子转运相关。具体目标是 1) 将 NPD 对低氯/异丙肾上腺素/阿米洛利的反应变化幅度与 CFTR 基因型相关联； 2）扩大对CF潜在替代标记与基因型之间关系的理解；并证实 NPD 测量作为有价值的研究工具的作用。发现 CF 部分但非全部特征的医生或根据 CFTR 基因型转诊患者。