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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 29;5:5.

PMID: 17394637 [PubMed - indexed for MEDLINE]

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 9;349(15):1433-41.

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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.

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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.

PMID: 10712334 [PubMed - indexed for MEDLINE]

Pharmacologic therapy for stop mutations: how much CFTR activity is enough?

Kerem E.

Curr Opin Pulm Med. 2004 Nov;10(6):547-52. Review.

PMID: 15510065 [PubMed - indexed for MEDLINE]

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. Epub 2007 Mar 8.

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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. 2002 Sep;80(9):595-604. Epub 2002 Jul 3.

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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. Epub 2007 Feb 8.

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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 13;284(11):6885-92. Epub 2009 Jan 9.

PMID: 19136563 [PubMed - indexed for MEDLINE]

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. 2006 Jul;84(7):573-82. Epub 2006 Mar 16.

PMID: 16541275 [PubMed - indexed for MEDLINE]

11:

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 30;372(9640):719-27. Epub 2008 Aug 20.

PMID: 18722008 [PubMed - indexed for MEDLINE]

12:

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.

PMID: 11786964 [PubMed - indexed for MEDLINE]

13:

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 3;6:25.

PMID: 17018149 [PubMed - indexed for MEDLINE]

14:

Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations.

Howard M, Frizzell RA, Bedwell DM.

Nat Med. 1996 Apr;2(4):467-9.

PMID: 8597960 [PubMed - indexed for MEDLINE]

15:

Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line.

Bedwell DM, Kaenjak A, Benos DJ, Bebok Z, Bubien JK, Hong J, Tousson A, Clancy JP, Sorscher EJ.

Nat Med. 1997 Nov;3(11):1280-4.

PMID: 9359706 [PubMed - indexed for MEDLINE]

16:

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. Epub 2007 May 31.

PMID: 17541014 [PubMed - indexed for MEDLINE]

17:

Variant cystic fibrosis phenotypes in the absence of CFTR mutations.

Groman JD, Meyer ME, Wilmott RW, Zeitlin PL, Cutting GR.

N Engl J Med. 2002 Aug 8;347(6):401-7.

PMID: 12167682 [PubMed - indexed for MEDLINE]

18:

Pharmacological induction of CFTR function in patients with cystic fibrosis: mutation-specific therapy.

Kerem E.

Pediatr Pulmonol. 2005 Sep;40(3):183-96. Review.

PMID: 15880796 [PubMed - indexed for MEDLINE]

19:

Cystic fibrosis transmembrane conductance regulator (CFTR) activity in nasal epithelial cells from cystic fibrosis patients with severe genotypes.

Andersson C, Dragomir A, Hjelte L, Roomans GM.

Clin Sci (Lond). 2002 Oct;103(4):417-24.

PMID: 12241542 [PubMed - indexed for MEDLINE]

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Cystic fibrosis transmembrane conductance regulator (CFTR)-mediated residual chloride secretion does not protect against early chronic Pseudomonas aeruginosa infection in F508del homozygous cystic fibrosis patients.

Derichs N, Mekus F, Bronsveld I, Bijman J, Veeze HJ, von der Hardt H, Tummler B, Ballmann M.

Pediatr Res. 2004 Jan;55(1):69-75. Epub 2003 Nov 6.

PMID: 14605249 [PubMed - indexed for MEDLINE]

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