CFTR

囊性纤维化（CF）是一种致命的遗传性肺部疾病。囊性纤维化传导调节器（CFTR）通道的突变已被证明可导致CF。CFTR是一种受调节的上皮氯通道，突变可通过多种机制导致通道活性降低。CFTR功能障碍导致慢性粘液阻塞、中性粒细胞炎症和CF气道细菌感染。这导致气道上皮细胞电解质转运缺陷，从而导致慢性肺部感染和过早死亡。因此，增加CFTR活性的化合物具有对CF和其他呼吸系统疾病的潜力，如慢性阻塞性肺疾病（COPD）或哮喘。

Noone, P.G. & Knowles, M.R. 2001. 'CFTR-opathies': disease phenotypes associated with cystic fibrosis transmembrane regulator gene mutations. Respiratory Research

Mall, M.A., & Hartl, D. 2014. CFTR: cystic fibrosis and beyond. European Respiratory Journal

Measuring CFTR Channels

CFTR Channels can be measured using automated patch clamp when expressed in cell lines, or using the Orbit instruments when they are recombinantly expressed in bilayers. The CFTR channel is an ABC transporter and can also be measured using the SSM-electrophysiology technique.

High throughput automated patch clamp

Parallel bilayer recordings

Transporter measurements

Application Notes

Publications using Instruments from Nanion

2021 - Cell engineering method using fluorogenic oligonucleotide signaling probes and flow cytometry

2020 - Targeting different binding sites in the CFTR structures allows to synergistically potentiate channel activity

2017 - Development of Automated Patch Clamp Technique to Investigate CFTR Chloride Channel Function

2017 - Buserelin alleviates chloride transport defect in human cystic fibrosis nasal epithelial cells

2014 - Improvement of Chloride Transport Defect by Gonadotropin-Releasing Hormone (GnRH) in Cystic Fibrosis Epithelial Cells