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Results: 1 to 20 of 110

Similar articles for PubMed (Select 22569626)

1.

The cystic-fibrosis-associated ΔF508 mutation confers post-transcriptional destabilization on the C. elegans ABC transporter PGP-3.

He L, Skirkanich J, Moronetti L, Lewis R, Lamitina T.

Dis Model Mech. 2012 Nov;5(6):930-9. doi: 10.1242/dmm.008987. Epub 2012 May 8.

2.

Intestinal apical polarity mediates regulation of TORC1 by glucosylceramide in C. elegans.

Zhu H, Sewell AK, Han M.

Genes Dev. 2015 Jun 15;29(12):1218-23. doi: 10.1101/gad.263483.115.

PMID:
26109047
3.

Rescuing trafficking mutants of the ATP binding cassette protein, ABCA4, with small molecule correctors as a treatment for Stargardt eye disease.

Sabirzhanova I, Lopes-Pacheco M, Rapino D, Grover R, Handa JT, Guggino WB, Cebotaru L.

J Biol Chem. 2015 Jun 19. pii: jbc.M115.647685. [Epub ahead of print]

4.

ΔF508 CFTR surface stability is regulated by DAB2 and CHIP-mediated ubiquitination in post-endocytic compartments.

Fu L, Rab A, Tang Lp, Bebok Z, Rowe SM, Bartoszewski R, Collawn JF.

PLoS One. 2015 Apr 16;10(4):e0123131. doi: 10.1371/journal.pone.0123131. eCollection 2015.

5.

Expression of three topologically distinct membrane proteins elicits unique stress response pathways in the yeast Saccharomyces cerevisiae.

Buck TM, Jordan R, Lyons-Weiler J, Adelman JL, Needham PG, Kleyman TR, Brodsky JL.

Physiol Genomics. 2015 Jun;47(6):198-214. doi: 10.1152/physiolgenomics.00101.2014. Epub 2015 Mar 10.

PMID:
25759377
6.

Activation of 3-phosphoinositide-dependent kinase 1 (PDK1) and serum- and glucocorticoid-induced protein kinase 1 (SGK1) by short-chain sphingolipid C4-ceramide rescues the trafficking defect of ΔF508-cystic fibrosis transmembrane conductance regulator (ΔF508-CFTR).

Caohuy H, Yang Q, Eudy Y, Ha TA, Xu AE, Glover M, Frizzell RA, Jozwik C, Pollard HB.

J Biol Chem. 2014 Dec 26;289(52):35953-68. doi: 10.1074/jbc.M114.598649. Epub 2014 Nov 10.

PMID:
25384981
7.

Cystic fibrosis transmembrane conductance regulator (CFTR) potentiators protect G551D but not ΔF508 CFTR from thermal instability.

Liu X, Dawson DC.

Biochemistry. 2014 Sep 9;53(35):5613-8. doi: 10.1021/bi501007v. Epub 2014 Aug 22.

PMID:
25148434
8.

Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis.

Cholon DM, Quinney NL, Fulcher ML, Esther CR Jr, Das J, Dokholyan NV, Randell SH, Boucher RC, Gentzsch M.

Sci Transl Med. 2014 Jul 23;6(246):246ra96. doi: 10.1126/scitranslmed.3008680.

9.

Restoration of NBD1 thermal stability is necessary and sufficient to correct ∆F508 CFTR folding and assembly.

He L, Aleksandrov AA, An J, Cui L, Yang Z, Brouillette CG, Riordan JR.

J Mol Biol. 2015 Jan 16;427(1):106-20. doi: 10.1016/j.jmb.2014.07.026. Epub 2014 Jul 30.

PMID:
25083918
10.

Conserved allosteric hot spots in the transmembrane domains of cystic fibrosis transmembrane conductance regulator (CFTR) channels and multidrug resistance protein (MRP) pumps.

Wei S, Roessler BC, Chauvet S, Guo J, Hartman JL 4th, Kirk KL.

J Biol Chem. 2014 Jul 18;289(29):19942-57. doi: 10.1074/jbc.M114.562116. Epub 2014 May 29.

PMID:
24876383
11.

Control of metazoan heme homeostasis by a conserved multidrug resistance protein.

Korolnek T, Zhang J, Beardsley S, Scheffer GL, Hamza I.

Cell Metab. 2014 Jun 3;19(6):1008-19. doi: 10.1016/j.cmet.2014.03.030. Epub 2014 May 15.

12.

Cystic fibrosis transmembrane conductance regulator-mRNA delivery: a novel alternative for cystic fibrosis gene therapy.

Bangel-Ruland N, Tomczak K, Fernández Fernández E, Leier G, Leciejewski B, Rudolph C, Rosenecker J, Weber WM.

J Gene Med. 2013 Nov-Dec;15(11-12):414-26. doi: 10.1002/jgm.2748.

PMID:
24123772
13.

RNF185 is a novel E3 ligase of endoplasmic reticulum-associated degradation (ERAD) that targets cystic fibrosis transmembrane conductance regulator (CFTR).

El Khouri E, Le Pavec G, Toledano MB, Delaunay-Moisan A.

J Biol Chem. 2013 Oct 25;288(43):31177-91. doi: 10.1074/jbc.M113.470500. Epub 2013 Sep 9.

14.

The silent codon change I507-ATC->ATT contributes to the severity of the ΔF508 CFTR channel dysfunction.

Lazrak A, Fu L, Bali V, Bartoszewski R, Rab A, Havasi V, Keiles S, Kappes J, Kumar R, Lefkowitz E, Sorscher EJ, Matalon S, Collawn JF, Bebok Z.

FASEB J. 2013 Nov;27(11):4630-45. doi: 10.1096/fj.13-227330. Epub 2013 Aug 1.

15.

Towards a rational combination therapy of cystic fibrosis: How cystamine restores the stability of mutant CFTR.

Villella VR, Esposito S, Maiuri MC, Raia V, Kroemer G, Maiuri L.

Autophagy. 2013 Sep;9(9):1431-4. doi: 10.4161/auto.25517. Epub 2013 Jun 25.

PMID:
23800975
16.

Effects of the gout-causing Q141K polymorphism and a CFTR ΔF508 mimicking mutation on the processing and stability of the ABCG2 protein.

Sarankó H, Tordai H, Telbisz Á, Özvegy-Laczka C, Erdős G, Sarkadi B, Hegedűs T.

Biochem Biophys Res Commun. 2013 Jul 19;437(1):140-5. doi: 10.1016/j.bbrc.2013.06.054. Epub 2013 Jun 22.

PMID:
23800412
17.

Control of cystic fibrosis transmembrane conductance regulator membrane trafficking: not just from the endoplasmic reticulum to the Golgi.

Farinha CM, Matos P, Amaral MD.

FEBS J. 2013 Sep;280(18):4396-406. doi: 10.1111/febs.12392. Epub 2013 Jul 5. Review.

PMID:
23773658
18.

Tgf-β1 inhibits Cftr biogenesis and prevents functional rescue of ΔF508-Cftr in primary differentiated human bronchial epithelial cells.

Snodgrass SM, Cihil KM, Cornuet PK, Myerburg MM, Swiatecka-Urban A.

PLoS One. 2013 May 9;8(5):e63167. doi: 10.1371/journal.pone.0063167. Print 2013.

19.

Mechanism-based corrector combination restores ΔF508-CFTR folding and function.

Okiyoneda T, Veit G, Dekkers JF, Bagdany M, Soya N, Xu H, Roldan A, Verkman AS, Kurth M, Simon A, Hegedus T, Beekman JM, Lukacs GL.

Nat Chem Biol. 2013 Jul;9(7):444-54. doi: 10.1038/nchembio.1253. Epub 2013 May 12.

20.

Targeting autophagy as a novel strategy for facilitating the therapeutic action of potentiators on ΔF508 cystic fibrosis transmembrane conductance regulator.

Luciani A, Villella VR, Esposito S, Gavina M, Russo I, Silano M, Guido S, Pettoello-Mantovani M, Carnuccio R, Scholte B, De Matteis A, Maiuri MC, Raia V, Luini A, Kroemer G, Maiuri L.

Autophagy. 2012 Nov;8(11):1657-72. doi: 10.4161/auto.21483. Epub 2012 Aug 9.

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