ABCMdb

PMID: 16787252

Verkman AS, Lukacs GL, Galietta LJ
CFTR chloride channel drug discovery--inhibitors as antidiarrheals and activators for therapy of cystic fibrosis.
Curr Pharm Des. 2006;12(18):2235-47., [PubMed]

Sentences

No. Mutations Sentence Comment

209 ABCC8 p.Ile152Leu To identify correctors of defective ∆F508-CFTR gating ('potentiators`), we designed a high-throughput screen using stably transfected FRT cells that co-express ∆F508-CFTR and the halide sensitive indicator YFP-H148Q/I152L [16] (Fig. 6A,B). Login to comment 221 ABCC7 p.Gly551Asp ABCC7 p.Gly1349Asp ABCC7 p.Asp1152His Phenylglycines had another interesting property in being able to activate other CFTR mutants including G551D, G1349D, and D1152H (Fig. 7C). Login to comment 232 ABCC8 p.Ile152Leu Cells co-expressing ∆F508-CFTR and the halide-sensitive fluorescent protein YFP-H148Q/I152L were grown for 24 h at 27 o C (to allow plasma membrane ∆F508-CFTR expression). Login to comment 233 ABCC8 p.Ile152Leu ABCC8 p.Ile152Leu After washing, test compounds (2.5 µM) and forskolin (20 µM) were added, and I- influx was assayed from the time course of YFP-H148Q/I152L fluorescence after adding Ito the external solution. B. Representative time courses of YFP-H148Q/I152L fluorescence in control wells (saline, negative control; 50 µM genistein, positive control) with examples of inactive and active test compounds. Login to comment 242 ABCC7 p.Gly1349Asp C. Apical membrane current in FRT cells expressing G1349D-CFTR showing responses to forskolin and genistein or PG-01. Login to comment 269 ABCC7 p.Pro574His epithelia, but did not correct a different temperature-sensitive CFTR mutant (P574H-CFTR) or a dopamine receptor mutant. Login to comment