CAMP STIMULATED SWEAT RATE IN CYSTIC FIBROSIS

囊性纤维化中 CAMP 刺激出汗率

• 批准号: 7604524
• 负责人: Pamela L. Zeitlin
• 金额: $ 0.04万
• 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/7604524
• 关键词: Adrenergic Agonists; Affect; Age; Camping; Chloride Ion; Chlorides; Cholinergic Agents; Class; Collection; Computer Retrieval of Information on Scientific Projects Database; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Development; Diagnostic tests; Duct (organ) structure; Funding; Gender; Goals; Grant; Heterozygote; Institution; Iontophoresis; Isotonic Exercise; Measurement; Mediating; Mutation; Pilocarpine; Production; Rate; Research; Research Personnel; Resources; Severity of illness; Skin; Source; Standards of Weights and Measures; Sweat; Sweat Glands; Sweat test; Sweating; Techniques; Testing; United States National Institutes of Health; base; cholinergic; cystic fibrosis patients

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. The goal of this project is the development of a cyclic-AMP(cAMP)-stimulated "sweat rate" test that will discriminate between fully functional, partially functional, and non-functional forms of the Cystic Fibrosis Transmembrane Conductance Regulator(CFTR). Sweat production can be stimulated by both cholinergic & adrenergic agonists. Collection of cholinergic stimulated sweat & the subsequent measurement of sweat chloride concentration is the basis of a standard diagnostic test for cystic fibrosis (CF), the pilocarpine iontophoresis sweat test. Stimulation of sweat production with pilocarpine leads to initial production of an isotonic secretion in the sweat gland. In non-CF patients, as the secretion traverses the sweat duct, chloride is reabsorbed leading to low concentrations of chloride in sweat as it appears on the skin. This chloride resorption is dependent on the presence of functional CFTR. In CF patients, who lack functional CFTR, the sweat chloride concentration remains high, & distinguishes most, but not all, CF from non-CF patients. However, this technique does not distinguish heterozygote carriers of CFTR mutations from non-carriers, nor does the sweat chloride concentration correlate with disease severity. The hypothesis is that measurement of cAMP-mediated sweat rates will distinguish among CF subjects, non-CF subjects, and non-affected carriers of CFTR mutations. A secondary hypothesis is that there will be a correlation between CFTR activity and sweat rate such that non-affected carriers of CFTR mutations will have intermediate sweat rates to CF and non-CF control subjects. Another secondary hypothesis is that different classes of CFTR mutations can be associated with different levels of sweat rate. Subjects of both genders and all ages are eligible for entry.

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。 该项目的目标是开发一种环磷酸腺苷 (cAMP) 刺激的“出汗率”测试，该测试将区分囊性纤维化跨膜电导调节器 (CFTR) 的完全功能、部分功能和非功能形式。 胆碱能和肾上腺素能激动剂都可以刺激出汗。 收集胆碱能刺激的汗液并随后测量汗液氯化物浓度是囊性纤维化 (CF) 标准诊断测试（毛果芸香碱离子电渗汗液测试）的基础。 用毛果芸香碱刺激出汗会导致汗腺初步产生等渗分泌物。 在非 CF 患者中，当分泌物穿过汗管时，氯化物被重新吸收，导致皮肤上出现的汗液中氯化物浓度较低。 这种氯离子吸收取决于功能性 CFTR 的存在。 在缺乏功能性 CFTR 的 CF 患者中，汗液氯化物浓度仍然很高，并且可以将大多数（但不是全部）CF 患者与非 CF 患者区分开来。 然而，该技术无法区分 CFTR 突变杂合子携带者和非携带者，汗液氯化物浓度也与疾病严重程度无关。 假设测量 cAMP 介导的出汗率将区分 CF 受试者、非 CF 受试者和未受影响的 CFTR 突变携带者。 第二个假设是，CFTR 活性和出汗率之间存在相关性，因此未受影响的 CFTR 突变携带者的出汗率与 CF 和非 CF 对照受试者处于中间水平。 另一个次要假设是，不同类别的 CFTR 突变可能与不同水平的出汗率相关。 所有性别和所有年龄段的受试者都有资格参赛。