Function of CFTR protein: ion transport. In: Cystic Fibrosis in the 21st Century, ed. Bush A, Alton EWFW, Davies JC, Griesenbach U & Jaffe A, pp. 38-44. Karger, Basel.Chen JH,Cai Z,Li H,Sheppard DN.Function of CFTR protein: ion transport. Cystic Fibrosis in the 21st Century ....
The assay is a valuable tool for investigation of CFTR molecular mechanisms, allowing accurate inferences on gating/permeation. In addition, by providing a two-dimensional characterization of the CFTR protein, it could better inform development of single-drug and precision therapies addressing the root...
The functional increases are similar to those obtained with tezacaftor/ivacaftor in F508del homozygous PDOs. Increased CFTR C- and B-band protein was observed in accordance with increased function. In addition, ELX-02 treatment of a G542X/G542X PDO results in a 5-fold increase in CFTR ...
Function of CFTR; Materials and met... O Baldursson,HA Berger,MJ Welsh,... 被引量: 0发表: 2019年 Stimulation of Elk1 Transcriptional Activity by Mitogen-activated Protein Kinases Is Negatively Regulated by Protein Phosphatase 2B (Calcineurin) Elk1 at phosphoserine 383, a site whose ...
protein called [...] chinese.eurekalert.org chinese.eurekalert.org 许多吸烟者患慢性阻塞性肺病,这是一组类似于囊性纤维化的病症,它是由肺的气道中的一种称为囊性纤维化跨膜电导调节因子(CFTR)的离子输运蛋白的功能缺陷导致的。 chinese.eurekalert.org ...
Lindgren gives examples of cystic fibrosis drugs that target particular mutations in the CFTR protein, and cholesterol-lowering drugs that inhibit PCSK9, mimicking a genetic variation in humans that lowers LDL cholesterol. “Genetic discoveries are enabling the development of drugs that target the root...
The combination of the CFTR modulators elexacaftor, tezacaftor, and ivacaftor (ETI) enables the effective rescue of CFTR function in people with the most prevalent F508del mutation. However, the functional restoration of rare CFTR variants remains unclear. Here, we use patient-derived i...
(CSD). A small (∼70 kDa) domain with high similarity to the ribonucleoprotein 1 RNA-binding motif. This domain is found in DNA- and RNA-binding proteins in bacteria, archaea and eukaryotes. Interfaces The solvent-accessible portions of a protein that are capable of binding a ligand — in...
The purpose of this study was to investigate the function of the protein bestrophin-1 (best-1). Previous studies have suggested that best-1 is either a Ca++-activated Cl- channel (ClCa) and/or that it is a regulator of Ca++ induced responses in the retinal pigment epithelium (RPE). My...
Protein ingestion (especially arginine), β-adrenergic stimulation, vigorous exercise, fasting and cholecystokinin secreted by duodenal cells also increase glucagon secretion. The main function of glucagon is to promote utilization of stored nutrients and its action principally involves the liver. Glucagon ...