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Items: 1 to 20 of 154

1.

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.

PMID:
11401894
2.

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.

3.

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.

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.

PMID:
10712334
5.

Ataluren (PTC124) induces cystic fibrosis transmembrane conductance regulator protein expression and activity in children with nonsense mutation cystic fibrosis.

Sermet-Gaudelus I, Boeck KD, Casimir GJ, Vermeulen F, Leal T, Mogenet A, Roussel D, Fritsch J, Hanssens L, Hirawat S, Miller NL, Constantine S, Reha A, Ajayi T, Elfring GL, Miller LL.

Am J Respir Crit Care Med. 2010 Nov 15;182(10):1262-72. doi: 10.1164/rccm.201001-0137OC. Epub 2010 Jul 9.

PMID:
20622033
6.

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.

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. Epub 2002 Jul 3.

PMID:
12226741
8.

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
9.

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. doi: 10.1016/S0140-6736(08)61168-X. Epub 2008 Aug 20.

PMID:
18722008
10.

Cystic fibrosis transmembrane conductance regulator channel dysfunction in non-cystic fibrosis bronchiectasis.

Bienvenu T, Sermet-Gaudelus I, Burgel PR, Hubert D, Crestani B, Bassinet L, Dusser D, Fajac I.

Am J Respir Crit Care Med. 2010 May 15;181(10):1078-84. doi: 10.1164/rccm.200909-1434OC. Epub 2010 Feb 18.

PMID:
20167849
11.

Relationship between airway ion transport and a mild pulmonary disease mutation in CFTR.

Walker LC, Venglarik CJ, Aubin G, Weatherly MR, McCarty NA, Lesnick B, Ruiz F, Clancy JP, Sorscher EJ.

Am J Respir Crit Care Med. 1997 May;155(5):1684-9.

PMID:
9154877
12.

Nasal potential difference measurements in patients with atypical cystic fibrosis.

Wilschanski M, Famini H, Strauss-Liviatan N, Rivlin J, Blau H, Bibi H, Bentur L, Yahav Y, Springer H, Kramer MR, Klar A, Ilani A, Kerem B, Kerem E.

Eur Respir J. 2001 Jun;17(6):1208-15.

13.

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
14.

Induction of a cAMP-stimulated chloride secretion in regenerating poorly differentiated airway epithelial cells by adenovirus-mediated CFTR gene transfer.

Dupuit F, Chinet T, Zahm JM, Pierrot D, Hinnrasky J, Kaplan H, Bonnet N, Puchelle E.

Hum Gene Ther. 1997 Aug 10;8(12):1439-50.

PMID:
9287144
15.

Quantitative fluorescence measurements of chloride secretion in native airway epithelium from CF and non-CF subjects.

Stern M, Munkonge FM, Caplen NJ, Sorgi F, Huang L, Geddes DM, Alton EW.

Gene Ther. 1995 Dec;2(10):766-74.

PMID:
8750017
16.

Measurement of nasal potential difference in young children with an equivocal sweat test following newborn screening for cystic fibrosis.

Sermet-Gaudelus I, Girodon E, Roussel D, Deneuville E, Bui S, Huet F, Guillot M, Aboutaam R, Renouil M, Munck A, des Georges M, Iron A, Thauvin-Robinet C, Fajac I, Lenoir G, Roussey M, Edelman A.

Thorax. 2010 Jun;65(6):539-44. doi: 10.1136/thx.2009.123422.

PMID:
20522854
17.

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. doi: 10.1165/rcmb.2013-0282OC.

18.

Chloride transport in nasal ciliated cells of cystic fibrosis heterozygotes.

Sermet-Gaudelus I, Déchaux M, Vallée B, Fajac A, Girodon E, Nguyen-Khoa T, Marianovski R, Hurbain I, Bresson JL, Lenoir G, Edelman A.

Am J Respir Crit Care Med. 2005 May 1;171(9):1026-31. Epub 2005 Feb 11.

PMID:
15709055
20.

CFTR Cl- channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis.

Hirtz S, Gonska T, Seydewitz HH, Thomas J, Greiner P, Kuehr J, Brandis M, Eichler I, Rocha H, Lopes AI, Barreto C, Ramalho A, Amaral MD, Kunzelmann K, Mall M.

Gastroenterology. 2004 Oct;127(4):1085-95.

PMID:
15480987

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