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Items: 7

1.

Structure-guided combination therapy to potently improve the function of mutant CFTRs.

Veit G, Xu H, Dreano E, Avramescu RG, Bagdany M, Beitel LK, Roldan A, Hancock MA, Lay C, Li W, Morin K, Gao S, Mak PA, Ainscow E, Orth AP, McNamara P, Edelman A, Frenkiel S, Matouk E, Sermet-Gaudelus I, Barnes WG, Lukacs GL.

Nat Med. 2018 Nov;24(11):1732-1742. doi: 10.1038/s41591-018-0200-x. Epub 2018 Oct 8.

2.

Hormonal vitamin D up-regulates tissue-specific PD-L1 and PD-L2 surface glycoprotein expression in humans but not mice.

Dimitrov V, Bouttier M, Boukhaled G, Salehi-Tabar R, Avramescu RG, Memari B, Hasaj B, Lukacs GL, Krawczyk CM, White JH.

J Biol Chem. 2017 Dec 15;292(50):20657-20668. doi: 10.1074/jbc.M117.793885. Epub 2017 Oct 23.

3.

Mutation-specific downregulation of CFTR2 variants by gating potentiators.

Avramescu RG, Kai Y, Xu H, Bidaud-Meynard A, Schnúr A, Frenkiel S, Matouk E, Veit G, Lukacs GL.

Hum Mol Genet. 2017 Dec 15;26(24):4873-4885. doi: 10.1093/hmg/ddx367.

4.

Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell.

Bagdany M, Veit G, Fukuda R, Avramescu RG, Okiyoneda T, Baaklini I, Singh J, Sovak G, Xu H, Apaja PM, Sattin S, Beitel LK, Roldan A, Colombo G, Balch W, Young JC, Lukacs GL.

Nat Commun. 2017 Aug 30;8(1):398. doi: 10.1038/s41467-017-00444-4.

5.

Correctors and Potentiators Rescue Function of the Truncated W1282X-Cystic Fibrosis Transmembrane Regulator (CFTR) Translation Product.

Haggie PM, Phuan PW, Tan JA, Xu H, Avramescu RG, Perdomo D, Zlock L, Nielson DW, Finkbeiner WE, Lukacs GL, Verkman AS.

J Biol Chem. 2017 Jan 20;292(3):771-785. doi: 10.1074/jbc.M116.764720. Epub 2016 Nov 28.

6.

From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations.

Veit G, Avramescu RG, Chiang AN, Houck SA, Cai Z, Peters KW, Hong JS, Pollard HB, Guggino WB, Balch WE, Skach WR, Cutting GR, Frizzell RA, Sheppard DN, Cyr DM, Sorscher EJ, Brodsky JL, Lukacs GL.

Mol Biol Cell. 2016 Feb 1;27(3):424-33. doi: 10.1091/mbc.E14-04-0935.

7.

Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression.

Veit G, Avramescu RG, Perdomo D, Phuan PW, Bagdany M, Apaja PM, Borot F, Szollosi D, Wu YS, Finkbeiner WE, Hegedus T, Verkman AS, Lukacs GL.

Sci Transl Med. 2014 Jul 23;6(246):246ra97. doi: 10.1126/scitranslmed.3008889.

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