524 related articles for article (PubMed ID: 17394637)
1. In vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study.
Sermet-Gaudelus I; Renouil M; Fajac A; Bidou L; Parbaille B; Pierrot S; Davy N; Bismuth E; Reinert P; Lenoir G; Lesure JF; Rousset JP; Edelman A
BMC Med; 2007 Mar; 5():5. PubMed ID: 17394637
[TBL] [Abstract][Full Text] [Related]
2. Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations.
Wilschanski M; Yahav Y; Yaacov Y; Blau H; Bentur L; Rivlin J; Aviram M; Bdolah-Abram T; Bebok Z; Shushi L; Kerem B; Kerem E
N Engl J Med; 2003 Oct; 349(15):1433-41. PubMed ID: 14534336
[TBL] [Abstract][Full Text] [Related]
3. Evidence that systemic gentamicin suppresses premature stop mutations in patients with cystic fibrosis.
Clancy JP; Bebök Z; Ruiz F; King C; Jones J; Walker L; Greer H; Hong J; Wing L; Macaluso M; Lyrene R; Sorscher EJ; Bedwell DM
Am J Respir Crit Care Med; 2001 Jun; 163(7):1683-92. PubMed ID: 11401894
[TBL] [Abstract][Full Text] [Related]
4. A pilot study of the effect of gentamicin on nasal potential difference measurements in cystic fibrosis patients carrying stop mutations.
Wilschanski M; Famini C; Blau H; Rivlin J; Augarten A; Avital A; Kerem B; Kerem E
Am J Respir Crit Care Med; 2000 Mar; 161(3 Pt 1):860-5. PubMed ID: 10712334
[TBL] [Abstract][Full Text] [Related]
5. Pharmacologic therapy for stop mutations: how much CFTR activity is enough?
Kerem E
Curr Opin Pulm Med; 2004 Nov; 10(6):547-52. PubMed ID: 15510065
[TBL] [Abstract][Full Text] [Related]
6. Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor.
Xue X; Mutyam V; Tang L; Biswas S; Du M; Jackson LA; Dai Y; Belakhov V; Shalev M; Chen F; Schacht J; J Bridges R; Baasov T; Hong J; Bedwell DM; Rowe SM
Am J Respir Cell Mol Biol; 2014 Apr; 50(4):805-16. PubMed ID: 24251786
[TBL] [Abstract][Full Text] [Related]
7. Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene.
Du M; Jones JR; Lanier J; Keeling KM; Lindsey JR; Tousson A; Bebök Z; Whitsett JA; Dey CR; Colledge WH; Evans MJ; Sorscher EJ; Bedwell DM
J Mol Med (Berl); 2002 Sep; 80(9):595-604. PubMed ID: 12226741
[TBL] [Abstract][Full Text] [Related]
8. Nonsense-mediated mRNA decay affects nonsense transcript levels and governs response of cystic fibrosis patients to gentamicin.
Linde L; Boelz S; Nissim-Rafinia M; Oren YS; Wilschanski M; Yaacov Y; Virgilis D; Neu-Yilik G; Kulozik AE; Kerem E; Kerem B
J Clin Invest; 2007 Mar; 117(3):683-92. PubMed ID: 17290305
[TBL] [Abstract][Full Text] [Related]
9. No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations.
Clancy JP; Rowe SM; Bebok Z; Aitken ML; Gibson R; Zeitlin P; Berclaz P; Moss R; Knowles MR; Oster RA; Mayer-Hamblett N; Ramsey B
Am J Respir Cell Mol Biol; 2007 Jul; 37(1):57-66. PubMed ID: 17347447
[TBL] [Abstract][Full Text] [Related]
10. Clinical doses of amikacin provide more effective suppression of the human CFTR-G542X stop mutation than gentamicin in a transgenic CF mouse model.
Du M; Keeling KM; Fan L; Liu X; Kovaçs T; Sorscher E; Bedwell DM
J Mol Med (Berl); 2006 Jul; 84(7):573-82. PubMed ID: 16541275
[TBL] [Abstract][Full Text] [Related]
11. Poly-L-aspartic acid enhances and prolongs gentamicin-mediated suppression of the CFTR-G542X mutation in a cystic fibrosis mouse model.
Du M; Keeling KM; Fan L; Liu X; Bedwell DM
J Biol Chem; 2009 Mar; 284(11):6885-92. PubMed ID: 19136563
[TBL] [Abstract][Full Text] [Related]
12. Novel Correctors and Potentiators Enhance Translational Readthrough in CFTR Nonsense Mutations.
Mutyam V; Sharma J; Li Y; Peng N; Chen J; Tang LP; Falk Libby E; Singh AK; Conrath K; Rowe SM
Am J Respir Cell Mol Biol; 2021 May; 64(5):604-616. PubMed ID: 33616476
[TBL] [Abstract][Full Text] [Related]
13. Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial.
Kerem E; Hirawat S; Armoni S; Yaakov Y; Shoseyov D; Cohen M; Nissim-Rafinia M; Blau H; Rivlin J; Aviram M; Elfring GL; Northcutt VJ; Miller LL; Kerem B; Wilschanski M
Lancet; 2008 Aug; 372(9640):719-27. PubMed ID: 18722008
[TBL] [Abstract][Full Text] [Related]
14. Suppression of CFTR premature termination codons and rescue of CFTR protein and function by the synthetic aminoglycoside NB54.
Rowe SM; Sloane P; Tang LP; Backer K; Mazur M; Buckley-Lanier J; Nudelman I; Belakhov V; Bebok Z; Schwiebert E; Baasov T; Bedwell DM
J Mol Med (Berl); 2011 Nov; 89(11):1149-61. PubMed ID: 21779978
[TBL] [Abstract][Full Text] [Related]
15. The CF-CIRC study: a French collaborative study to assess the accuracy of cystic fibrosis diagnosis in neonatal screening.
Sermet-Gaudelus I; Roussel D; Bui S; Deneuville E; Huet F; Reix P; Bellon G; Lenoir G; Edelman A
BMC Pediatr; 2006 Oct; 6():25. PubMed ID: 17018149
[TBL] [Abstract][Full Text] [Related]
16. Limited premature termination codon suppression by read-through agents in cystic fibrosis intestinal organoids.
Zomer-van Ommen DD; Vijftigschild LA; Kruisselbrink E; Vonk AM; Dekkers JF; Janssens HM; de Winter-de Groot KM; van der Ent CK; Beekman JM
J Cyst Fibros; 2016 Mar; 15(2):158-62. PubMed ID: 26255232
[TBL] [Abstract][Full Text] [Related]
17. Restoration of W1282X CFTR activity by enhanced expression.
Rowe SM; Varga K; Rab A; Bebok Z; Byram K; Li Y; Sorscher EJ; Clancy JP
Am J Respir Cell Mol Biol; 2007 Sep; 37(3):347-56. PubMed ID: 17541014
[TBL] [Abstract][Full Text] [Related]
18. Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations.
Howard M; Frizzell RA; Bedwell DM
Nat Med; 1996 Apr; 2(4):467-9. PubMed ID: 8597960
[TBL] [Abstract][Full Text] [Related]
19. Identification of Compounds That Promote Readthrough of Premature Termination Codons in the CFTR.
Smith E; Dukovski D; Shumate J; Scampavia L; Miller JP; Spicer TP
SLAS Discov; 2021 Feb; 26(2):205-215. PubMed ID: 33016182
[TBL] [Abstract][Full Text] [Related]
20. Correction of CFTR malfunction and stimulation of Ca-activated Cl channels restore HCO3- secretion in cystic fibrosis bile ductular cells.
Zsembery A; Jessner W; Sitter G; Spirlí C; Strazzabosco M; Graf J
Hepatology; 2002 Jan; 35(1):95-104. PubMed ID: 11786964
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
