Suppression of CFTR premature termination codons and rescue of CFTR protein and function by the synthetic aminoglycoside NB54

Journal of Molecular Medicine

Volumn 89, Issue 11, 2011, Pages 1149-1161

  Rowe, Steven M ^a,b,c,e,h   Sloane, Peter ^a,e   Tang, Li Ping ^a,e   Backer, Kyle ^a,e   Mazur, Marina ^e   Buckley Lanier, Jessica ^d   Nudelman, Igor ^f   Belakhov, Valery ^f   Bebok, Zsuzsa ^c,e   Schwiebert, Erik ^g   Baasov, Timor ^f   Bedwell, David M ^c,d,e  

^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 TECHNION ISRAEL INSTITUTE OF TECHNOLOGY   (Israel)

^g Innovation Depot   (United States)

^h UNIVERSITY OF ALABAMA AT BIRMINGHAM   (United States)

Author keywords

Aminoglycosides; Cystic fibrosis transmembrane conductance regulator; Premature termination codons; Primary human bronchial epithelial cells; Short circuit current

Indexed keywords

AMINOGLYCOSIDE DERIVATIVE; AMINOGLYCOSIDE NB30; AMINOGLYCOSIDE NB54; COMPLEMENTARY DNA; GENTAMICIN; HALIDE; TRANSMEMBRANE CONDUCTANCE REGULATOR; UNCLASSIFIED DRUG;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; CELL MEMBRANE CONDUCTANCE; CLINICAL PROTOCOL; CODON; CONTROLLED STUDY; CYSTIC FIBROSIS; DRUG CYTOTOXICITY; DRUG STRUCTURE; DRUG TOLERABILITY; EPITHELIUM CELL; GENOTYPE; HUMAN; HUMAN CELL; IMMUNOHISTOCHEMISTRY; IN VITRO STUDY; MOUSE; NONHUMAN; PREMATURE TERMINATION CODON; PROTEIN ANALYSIS; PROTEIN FUNCTION;

AMINOGLYCOSIDES; ANIMALS; CODON, TERMINATOR; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; GENTAMICINS; HELA CELLS; HUMANS; ION TRANSPORT; MICE; MICE, INBRED CFTR; MICE, KNOCKOUT; PROTEIN SYNTHESIS INHIBITORS; RESPIRATORY MUCOSA;

EID: 80755133472     PISSN: 09462716     EISSN: 14321440     Source Type: Journal    
DOI: 10.1007/s00109-011-0787-6     Document Type: Article

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