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.

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。鼻电位差测量是CF的一种新的诊断工具。本研究的假设是CFTR的IV类或V类突变将与NPD中的氯化物运输有关。具体目的是：1)将NPD对低氯化物/异丙肾上腺素/氨酰的反应变化幅度与CFTR基因型相关联；2)扩大对CF中潜在替代标记物与基因型之间关系的理解；并证实NPD测量作为一种有价值的研究工具的作用。患者由发现CF的一些但不是全部特征的医生转诊，或根据CFTR基因型。