195 related articles for article (PubMed ID: 26879107)
1. Rescue of defective ATP8B1 trafficking by CFTR correctors as a therapeutic strategy for familial intrahepatic cholestasis.
van der Woerd WL; Wichers CG; Vestergaard AL; Andersen JP; Paulusma CC; Houwen RH; van de Graaf SF
J Hepatol; 2016 Jun; 64(6):1339-47. PubMed ID: 26879107
[TBL] [Abstract][Full Text] [Related]
2. Folding defects in P-type ATP 8B1 associated with hereditary cholestasis are ameliorated by 4-phenylbutyrate.
van der Velden LM; Stapelbroek JM; Krieger E; van den Berghe PV; Berger R; Verhulst PM; Holthuis JC; Houwen RH; Klomp LW; van de Graaf SF
Hepatology; 2010 Jan; 51(1):286-96. PubMed ID: 19918981
[TBL] [Abstract][Full Text] [Related]
3. The phospholipid flippase ATP8B1 mediates apical localization of the cystic fibrosis transmembrane regulator.
van der Mark VA; de Jonge HR; Chang JC; Ho-Mok KS; Duijst S; Vidović D; Carlon MS; Oude Elferink RP; Paulusma CC
Biochim Biophys Acta; 2016 Sep; 1863(9):2280-8. PubMed ID: 27301931
[TBL] [Abstract][Full Text] [Related]
4. Differential effects of progressive familial intrahepatic cholestasis type 1 and benign recurrent intrahepatic cholestasis type 1 mutations on canalicular localization of ATP8B1.
Folmer DE; van der Mark VA; Ho-Mok KS; Oude Elferink RP; Paulusma CC
Hepatology; 2009 Nov; 50(5):1597-605. PubMed ID: 19731236
[TBL] [Abstract][Full Text] [Related]
5. A flippase-independent function of ATP8B1, the protein affected in familial intrahepatic cholestasis type 1, is required for apical protein expression and microvillus formation in polarized epithelial cells.
Verhulst PM; van der Velden LM; Oorschot V; van Faassen EE; Klumperman J; Houwen RH; Pomorski TG; Holthuis JC; Klomp LW
Hepatology; 2010 Jun; 51(6):2049-60. PubMed ID: 20512993
[TBL] [Abstract][Full Text] [Related]
6. Optimal correction of distinct CFTR folding mutants in rectal cystic fibrosis organoids.
Dekkers JF; Gogorza Gondra RA; Kruisselbrink E; Vonk AM; Janssens HM; de Winter-de Groot KM; van der Ent CK; Beekman JM
Eur Respir J; 2016 Aug; 48(2):451-8. PubMed ID: 27103391
[TBL] [Abstract][Full Text] [Related]
7. Effect of CFTR correctors on the traffic and the function of intracellularly retained ABCB4 variants.
Ben Saad A; Vauthier V; Tóth Á; Janaszkiewicz A; Durand-Schneider AM; Bruneau A; Delaunay JL; Lapalus M; Mareux E; Garcin I; Gonzales E; Housset C; Aït-Slimane T; Jacquemin E; Di Meo F; Falguières T
Liver Int; 2021 Jun; 41(6):1344-1357. PubMed ID: 33650203
[TBL] [Abstract][Full Text] [Related]
8. Cystic Fibrosis: Proteostatic correctors of CFTR trafficking and alternative therapeutic targets.
Hanrahan JW; Sato Y; Carlile GW; Jansen G; Young JC; Thomas DY
Expert Opin Ther Targets; 2019 Aug; 23(8):711-724. PubMed ID: 31169041
[No Abstract] [Full Text] [Related]
9. The HDAC inhibitor SAHA does not rescue CFTR membrane expression in Cystic Fibrosis.
Bergougnoux A; Petit A; Knabe L; Bribes E; Chiron R; De Sario A; Claustres M; Molinari N; Vachier I; Taulan-Cadars M; Bourdin A
Int J Biochem Cell Biol; 2017 Jul; 88():124-132. PubMed ID: 28478266
[TBL] [Abstract][Full Text] [Related]
10. Searching for combinations of small-molecule correctors to restore f508del-cystic fibrosis transmembrane conductance regulator function and processing.
Boinot C; Jollivet Souchet M; Ferru-Clément R; Becq F
J Pharmacol Exp Ther; 2014 Sep; 350(3):624-34. PubMed ID: 24970923
[TBL] [Abstract][Full Text] [Related]
11. Altered hepatobiliary gene expressions in PFIC1: ATP8B1 gene defect is associated with CFTR downregulation.
Demeilliers C; Jacquemin E; Barbu V; Mergey M; Paye F; Fouassier L; Chignard N; Housset C; Lomri NE
Hepatology; 2006 May; 43(5):1125-34. PubMed ID: 16628629
[TBL] [Abstract][Full Text] [Related]
12. Combination of Correctors Rescues CFTR Transmembrane-Domain Mutants by Mitigating their Interactions with Proteostasis.
Lopes-Pacheco M; Boinot C; Sabirzhanova I; Rapino D; Cebotaru L
Cell Physiol Biochem; 2017; 41(6):2194-2210. PubMed ID: 28448979
[TBL] [Abstract][Full Text] [Related]
13. Increasing the Endoplasmic Reticulum Pool of the F508del Allele of the Cystic Fibrosis Transmembrane Conductance Regulator Leads to Greater Folding Correction by Small Molecule Therapeutics.
Chung WJ; Goeckeler-Fried JL; Havasi V; Chiang A; Rowe SM; Plyler ZE; Hong JS; Mazur M; Piazza GA; Keeton AB; White EL; Rasmussen L; Weissman AM; Denny RA; Brodsky JL; Sorscher EJ
PLoS One; 2016; 11(10):e0163615. PubMed ID: 27732613
[TBL] [Abstract][Full Text] [Related]
14. Unravelling druggable signalling networks that control F508del-CFTR proteostasis.
Hegde RN; Parashuraman S; Iorio F; Ciciriello F; Capuani F; Carissimo A; Carrella D; Belcastro V; Subramanian A; Bounti L; Persico M; Carlile G; Galietta L; Thomas DY; Di Bernardo D; Luini A
Elife; 2015 Dec; 4():. PubMed ID: 26701908
[TBL] [Abstract][Full Text] [Related]
15. Regulated recycling of mutant CFTR is partially restored by pharmacological treatment.
Holleran JP; Zeng J; Frizzell RA; Watkins SC
J Cell Sci; 2013 Jun; 126(Pt 12):2692-703. PubMed ID: 23572510
[TBL] [Abstract][Full Text] [Related]
16. Compounds that correct F508del-CFTR trafficking can also correct other protein trafficking diseases: an in vitro study using cell lines.
Sampson HM; Lam H; Chen PC; Zhang D; Mottillo C; Mirza M; Qasim K; Shrier A; Shyng SL; Hanrahan JW; Thomas DY
Orphanet J Rare Dis; 2013 Jan; 8():11. PubMed ID: 23316740
[TBL] [Abstract][Full Text] [Related]
17. Correctors Rescue CFTR Mutations in Nucleotide-Binding Domain 1 (NBD1) by Modulating Proteostasis.
Lopes-Pacheco M; Sabirzhanova I; Rapino D; Morales MM; Guggino WB; Cebotaru L
Chembiochem; 2016 Mar; 17(6):493-505. PubMed ID: 26864378
[TBL] [Abstract][Full Text] [Related]
18. The lipid flippase heterodimer ATP8B1-CDC50A is essential for surface expression of the apical sodium-dependent bile acid transporter (SLC10A2/ASBT) in intestinal Caco-2 cells.
van der Mark VA; de Waart DR; Ho-Mok KS; Tabbers MM; Voogt HW; Oude Elferink RP; Knisely AS; Paulusma CC
Biochim Biophys Acta; 2014 Dec; 1842(12 Pt A):2378-86. PubMed ID: 25239307
[TBL] [Abstract][Full Text] [Related]
19. A small molecule that binds to an ATPase domain of Hsc70 promotes membrane trafficking of mutant cystic fibrosis transmembrane conductance regulator.
Cho HJ; Gee HY; Baek KH; Ko SK; Park JM; Lee H; Kim ND; Lee MG; Shin I
J Am Chem Soc; 2011 Dec; 133(50):20267-76. PubMed ID: 22074182
[TBL] [Abstract][Full Text] [Related]
20. Progressive familial intrahepatic cholestasis type 1.
Paulusma CC; Elferink RP; Jansen PL
Semin Liver Dis; 2010 May; 30(2):117-24. PubMed ID: 20422494
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]