Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor

American Journal of Respiratory Cell and Molecular Biology

Volumn 50, Issue 4, 2014, Pages 805-816

  Xue, Xiaojiao ^a,f   Mutyam, Venkateshwar ^b   Tang, Liping ^b   Biswas, Silpak ^b   Du, Ming ^e,f   Jackson, Laura A ^b   Dai, Yanying ^e,f   Belakhov, Valery ^g   Shalev, Moran ^g   Chen, Fuquan ^h   Schacht, Jochen ^h   Bridges, Robert J ⁱ   Baasov, Timor ^g   Hong, Jeong ^d,f   Bedwell, David M ^a,d,e,f   Rowe, Steven M ^b,c,d,f  

^a UNIVERSITY OF ALABAMA AT BIRMINGHAM   (United States)

^b University of Alabama at Birmingham   (United States)

^c University of Alabama at Birmingham   (United States)

^d UNIVERSITY OF ALABAMA AT BIRMINGHAM   (United States)

^e University of Alabama at Birmingham   (United States)

^f UNIVERSITY OF ALABAMA AT BIRMINGHAM   (United States)

^g TECHNION ISRAEL INSTITUTE OF TECHNOLOGY   (Israel)

^h UNIVERSITY OF MICHIGAN MEDICAL SCHOOL   (United States)

ⁱ ROSALIND FRANKLIN UNIVERSITY OF MEDICINE AND SCIENCE   (United States)

Author keywords

Aminoglycosides; Cystic fibrosis transmembrane conductance regulator; Ivacaftor; Nonsense mutations; Translational readthrough

Indexed keywords

AMINOGLYCOSIDE ANTIBIOTIC AGENT; COMPLEMENTARY DNA; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; DSB NB 124; GENTAMICIN; IVACAFTOR; NB 124; UNCLASSIFIED DRUG;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BRONCHUS MUCOSA; CELL ACTIVITY; CHLORIDE TRANSPORT; COCHLEA; CODON; CONTROLLED STUDY; CYSTIC FIBROSIS; DRUG BLOOD LEVEL; DRUG CYTOTOXICITY; DRUG DOSE INCREASE; DRUG EFFICACY; EPITHELIUM CELL; EXPLANT; FISCHER 344 RAT; GENE EXPRESSION; GENE REPRESSION; GENE SEQUENCE; GENETIC TRANSDUCTION; HAIR CELL; HUMAN; HUMAN CELL; LUCIFERASE ASSAY; MOUSE; NONHUMAN; NONSENSE MUTATION; OTOTOXICITY; PROTEIN EXPRESSION; PROTEIN FUNCTION; RAT; STOP CODON; THYROID CELL; TRANSGENE; WILD TYPE;

AMINOGLYCOSIDES; AMINOPHENOLS; ANIMALS; BIOLOGICAL TRANSPORT; CELL LINE; CHLORIDES; CODON, NONSENSE; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; DISEASE MODELS, ANIMAL; DRUG SYNERGISM; GENES, REPORTER; HUMANS; LUCIFERASES; MICE; MICE, INBRED CFTR; MICE, TRANSGENIC; ORGAN OF CORTI; QUINOLONES; RATS; RATS, INBRED F344; TIME FACTORS; TRANSFECTION;

EID: 84898715557     PISSN: 10441549     EISSN: 15354989     Source Type: Journal    
DOI: 10.1165/rcmb.2013-0282OC     Document Type: Article

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