ABCMdb

PMID: 10223721

Mansoura MK, Biwersi J, Ashlock MA, Verkman AS
Fluorescent chloride indicators to assess the efficacy of CFTR cDNA delivery.
Hum Gene Ther. 1999 Apr 10;10(6):861-75., 1999-04-10 [PubMed]

Sentences

No. Mutations Sentence Comment

249 ABCC7 p.Gly551Asp ABCC7 p.Gly542* Cl2 INDICA TOR APPLICATION TO STUDY CFTR FUNCTION (CONT`D ) Cell or tissue Protocol Cell SPQ Quench Findings/significance type Descriptiona loading ion Ref. Deletion of 19 residues HEK 293- Embryonic kidney cell line Passive Cl Xie et al. in the loop between EBNA 5 mM (1995) TM4 and TM5 12±18 hr eliminated Cl transport Coexpression of the N HeLa Cervical carcinom a (epithelial) Passive I Ostedgaard and C halves of CFTR 10 mM et al. resulted in functional 12±18 hr (1997) Cl channels C orrection of C F phenotype by pharmacological maneuvers Increased [IBMX] L cells Mouse fibroblasts Hypotonic I Yang et al. improved D F508 12 hr (1993) response; reduced temperature improved D F508 and G551D Transfer of purified CHO-CFTR Chinese hamster ovary Passive I Marshall CFTR protein induced FRT-CFTR Fisher rat thyroid epithelial 10 mM et al. Cl2 transport LLCPK1 Pig kidney epithelial 12±18 hr (1994) Overexpression of CFPAC-1 Pancreatic duct adenocarcinoma Passive or I Cheng et al. D F508 with sodium JME/CF15 (D F/D F) Hypotonic (1995) butyrate resulted in 1° culture Transformed CF airway 10 mM detectable Cl2 transport epithelial CF airway epithelial Chemical chaperones Swiss 3T3 Mouse embryo fibroblast Passive Cl Brown et al. (glycerol, D2O, TMAO) 5 mM (1996) corrected the CF 12±18 hr phenotype Aminoglycosid e antibiotics HeLa Cervical carcinom a (epithelial) Hypotonic I Howard et al. overcam e premature 10 mM (1996a) stop codons and 10 min corrected CF phenotype Deoxyspergualin C127-CFTR Mouse mammary epithelial Hypotonic I Jiang et al. delivered D F508 to JME/CF15 Transformed CF airway 10 mM (1998) plasma membrane and IBE-1 epithelial 4 min partially restored Cl2 Transformed intrahepatic transport (D F/G542X) Abbreviations: wt, wildtype; PKA, protein kinase A; TM, transmembrane ; TMAO, trimethylamine -N-oxide. Login to comment 252 ABCC7 p.Arg553* ABCC7 p.Gly542* The efficacy of aminoglycoside s in promoting read-through in the CFTR nonsense mutations G542X and R553X was shown using Cl2 indicators (Howard et al., 1996a). Login to comment