CAMP STIMULATED SWEAT RATE IN CYSTIC FIBROSIS

囊性纤维化中 CAMP 刺激出汗率

• 批准号: 6297531
• 负责人: Ronald C Rubenstein
• 金额: $ 0.23万
• 依托单位: JOHNS HOPKINS UNIVERSITY
• 依托单位国家: 美国
• 财政年份: 1998
• 资助国家: 美国
• 起止时间: 1998-12-01 至 1999-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/6297531
• 关键词: biomarker; chloride channels; clinical research; cyclic AMP; cystic fibrosis; diagnosis design /evaluation; diagnosis quality /standard; electrolyte balance; human subject; iontophoresis therapy; metabolism disorder diagnosis; sweat

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 cholride 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.

该项目的目标是开发一种循环amp (cAMP)-