161 related articles for article (PubMed ID: 33753261)
1. The development of highly potent and selective small molecule correctors of Z α
Liddle J; Pearce AC; Arico-Muendel C; Belyanskaya S; Brewster A; Brown M; Chung CW; Denis A; Dodic N; Dossang A; Eddershaw P; Klimaszewska D; Haq I; Holmes DS; Jagger A; Jakhria T; Jigorel E; Lind K; Messer J; Neu M; Olszewski A; Ronzoni R; Rowedder J; Rüdiger M; Skinner S; Smith KJ; Trottet L; Uings I; Zhu Z; Irving JA; Lomas DA
Bioorg Med Chem Lett; 2021 Jun; 41():127973. PubMed ID: 33753261
[TBL] [Abstract][Full Text] [Related]
2. Development of a small molecule that corrects misfolding and increases secretion of Z α
Lomas DA; Irving JA; Arico-Muendel C; Belyanskaya S; Brewster A; Brown M; Chung CW; Dave H; Denis A; Dodic N; Dossang A; Eddershaw P; Klimaszewska D; Haq I; Holmes DS; Hutchinson JP; Jagger AM; Jakhria T; Jigorel E; Liddle J; Lind K; Marciniak SJ; Messer J; Neu M; Olszewski A; Ordonez A; Ronzoni R; Rowedder J; Rüdiger M; Skinner S; Smith KJ; Terry R; Trottet L; Uings I; Wilson S; Zhu Z; Pearce AC
EMBO Mol Med; 2021 Mar; 13(3):e13167. PubMed ID: 33512066
[TBL] [Abstract][Full Text] [Related]
3. A transgenic zebrafish model of hepatocyte function in human Z α1-antitrypsin deficiency.
Yip E; Giousoh A; Fung C; Wilding B; Prakash MD; Williams C; Verkade H; Bryson-Richardson RJ; Bird PI
Biol Chem; 2019 Nov; 400(12):1603-1616. PubMed ID: 31091192
[TBL] [Abstract][Full Text] [Related]
4. Retarded protein folding of the human Z-type α₁-antitrypsin variant is suppressed by Cpr2p.
Jung CH; Kim YH; Lee K; Im H
Biochem Biophys Res Commun; 2014 Feb; 445(1):191-5. PubMed ID: 24502947
[TBL] [Abstract][Full Text] [Related]
5. Crystallographic and cellular characterisation of two mechanisms stabilising the native fold of alpha1-antitrypsin: implications for disease and drug design.
Gooptu B; Miranda E; Nobeli I; Mallya M; Purkiss A; Brown SC; Summers C; Phillips RL; Lomas DA; Barrett TE
J Mol Biol; 2009 Apr; 387(4):857-68. PubMed ID: 19232354
[TBL] [Abstract][Full Text] [Related]
6. The selective advantage of alpha1-antitrypsin deficiency.
Lomas DA
Am J Respir Crit Care Med; 2006 May; 173(10):1072-7. PubMed ID: 16439713
[TBL] [Abstract][Full Text] [Related]
7. A single-chain variable fragment intrabody prevents intracellular polymerization of Z α1-antitrypsin while allowing its antiproteinase activity.
Ordóñez A; Pérez J; Tan L; Dickens JA; Motamedi-Shad N; Irving JA; Haq I; Ekeowa U; Marciniak SJ; Miranda E; Lomas DA
FASEB J; 2015 Jun; 29(6):2667-78. PubMed ID: 25757566
[TBL] [Abstract][Full Text] [Related]
8. Regulator of G Signaling 16 is a marker for the distinct endoplasmic reticulum stress state associated with aggregated mutant alpha1-antitrypsin Z in the classical form of alpha1-antitrypsin deficiency.
Hidvegi T; Mirnics K; Hale P; Ewing M; Beckett C; Perlmutter DH
J Biol Chem; 2007 Sep; 282(38):27769-80. PubMed ID: 17635928
[TBL] [Abstract][Full Text] [Related]
9. The molecular species responsible for α
Ronzoni R; Heyer-Chauhan N; Fra A; Pearce AC; Rüdiger M; Miranda E; Irving JA; Lomas DA
FEBS J; 2021 Apr; 288(7):2222-2237. PubMed ID: 33058391
[TBL] [Abstract][Full Text] [Related]
10. The endoplasmic reticulum degradation pathway for mutant secretory proteins alpha1-antitrypsin Z and S is distinct from that for an unassembled membrane protein.
Teckman JH; Perlmutter DH
J Biol Chem; 1996 May; 271(22):13215-20. PubMed ID: 8662752
[TBL] [Abstract][Full Text] [Related]
11. High-resolution ex vivo NMR spectroscopy of human Z α
Jagger AM; Waudby CA; Irving JA; Christodoulou J; Lomas DA
Nat Commun; 2020 Dec; 11(1):6371. PubMed ID: 33311470
[TBL] [Abstract][Full Text] [Related]
12. Changes of endoplasmic reticulum chaperone complexes, redox state, and impaired protein disulfide reductase activity in misfolding alpha1-antitrypsin transgenic mice.
Papp E; Száraz P; Korcsmáros T; Csermely P
FASEB J; 2006 May; 20(7):1018-20. PubMed ID: 16571774
[TBL] [Abstract][Full Text] [Related]
13. Therapeutic targeting of misfolding and conformational change in α1-antitrypsin deficiency.
Nyon MP; Gooptu B
Future Med Chem; 2014 Jun; 6(9):1047-65. PubMed ID: 25068987
[TBL] [Abstract][Full Text] [Related]
14. Alpha1-antitrypsin deficiency: liver disease associated with retention of a mutant secretory glycoprotein in the endoplasmic reticulum.
Perlmutter DH
Methods Mol Biol; 2003; 232():39-56. PubMed ID: 12840538
[No Abstract] [Full Text] [Related]
15. Sequestration of mutated alpha1-antitrypsin into inclusion bodies is a cell-protective mechanism to maintain endoplasmic reticulum function.
Granell S; Baldini G; Mohammad S; Nicolin V; Narducci P; Storrie B; Baldini G
Mol Biol Cell; 2008 Feb; 19(2):572-86. PubMed ID: 18045994
[TBL] [Abstract][Full Text] [Related]
16. Characterising the association of latency with α(1)-antitrypsin polymerisation using a novel monoclonal antibody.
Tan L; Perez J; Mela M; Miranda E; Burling KA; Rouhani FN; DeMeo DL; Haq I; Irving JA; Ordóñez A; Dickens JA; Brantly M; Marciniak SJ; Alexander GJ; Gooptu B; Lomas DA
Int J Biochem Cell Biol; 2015 Jan; 58():81-91. PubMed ID: 25462157
[TBL] [Abstract][Full Text] [Related]
17. Activation of endoplasmic reticulum-specific stress responses associated with the conformational disease Z alpha 1-antitrypsin deficiency.
Lawless MW; Greene CM; Mulgrew A; Taggart CC; O'Neill SJ; McElvaney NG
J Immunol; 2004 May; 172(9):5722-6. PubMed ID: 15100318
[TBL] [Abstract][Full Text] [Related]
18. The endoplasmic reticulum remains functionally connected by vesicular transport after its fragmentation in cells expressing Z-α1-antitrypsin.
Dickens JA; Ordóñez A; Chambers JE; Beckett AJ; Patel V; Malzer E; Dominicus CS; Bradley J; Peden AA; Prior IA; Lomas DA; Marciniak SJ
FASEB J; 2016 Dec; 30(12):4083-4097. PubMed ID: 27601439
[TBL] [Abstract][Full Text] [Related]
19. A kinetic mechanism for the polymerization of alpha1-antitrypsin.
Dafforn TR; Mahadeva R; Elliott PR; Sivasothy P; Lomas DA
J Biol Chem; 1999 Apr; 274(14):9548-55. PubMed ID: 10092640
[TBL] [Abstract][Full Text] [Related]
20. Update on alpha-1 antitrypsin deficiency: New therapies.
Lomas DA; Hurst JR; Gooptu B
J Hepatol; 2016 Aug; 65(2):413-24. PubMed ID: 27034252
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
