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Alpha1-antitrypsin deficiency--a model for conformational diseases.
Carrell RW, Lomas DA. Carrell RW, et al. Among authors: lomas da. N Engl J Med. 2002 Jan 3;346(1):45-53. doi: 10.1056/NEJMra010772. N Engl J Med. 2002. PMID: 11778003 Review. No abstract available.
New Therapeutic Targets for Alpha-1 Antitrypsin Deficiency.
Lomas DA. Lomas DA. Chronic Obstr Pulm Dis. 2018 Aug 6;5(4):233-243. doi: 10.15326/jcopdf.5.4.2017.0165. Chronic Obstr Pulm Dis. 2018. PMID: 30723781 Free PMC article.
hiPSC hepatocyte model demonstrates the role of unfolded protein response and inflammatory networks in α1-antitrypsin deficiency.
Segeritz CP, Rashid ST, de Brito MC, Serra MP, Ordonez A, Morell CM, Kaserman JE, Madrigal P, Hannan NRF, Gatto L, Tan L, Wilson AA, Lilley K, Marciniak SJ, Gooptu B, Lomas DA, Vallier L. Segeritz CP, et al. Among authors: lomas da. J Hepatol. 2018 Oct;69(4):851-860. doi: 10.1016/j.jhep.2018.05.028. Epub 2018 Jun 5. J Hepatol. 2018. PMID: 29879455 Free PMC article.
Liver: Taking out the JuNK to treat α1-antitrypsin deficiency.
Rashid ST, Lomas DA. Rashid ST, et al. Among authors: lomas da. Nat Rev Gastroenterol Hepatol. 2017 Apr;14(4):201-202. doi: 10.1038/nrgastro.2017.22. Epub 2017 Mar 8. Nat Rev Gastroenterol Hepatol. 2017. PMID: 28270697 No abstract available.
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. Ordóñez A, et al. Among authors: lomas da. FASEB J. 2015 Jun;29(6):2667-78. doi: 10.1096/fj.14-267351. Epub 2015 Mar 10. FASEB J. 2015. PMID: 25757566 Free PMC article.
An integrative approach combining ion mobility mass spectrometry, X-ray crystallography, and nuclear magnetic resonance spectroscopy to study the conformational dynamics of α1 -antitrypsin upon ligand binding.
Nyon MP, Prentice T, Day J, Kirkpatrick J, Sivalingam GN, Levy G, Haq I, Irving JA, Lomas DA, Christodoulou J, Gooptu B, Thalassinos K. Nyon MP, et al. Among authors: lomas da. Protein Sci. 2015 Aug;24(8):1301-12. doi: 10.1002/pro.2706. Epub 2015 Jul 14. Protein Sci. 2015. PMID: 26011795 Free PMC article.
The molecular and cellular pathology of α₁-antitrypsin deficiency.
Gooptu B, Dickens JA, Lomas DA. Gooptu B, et al. Among authors: lomas da. Trends Mol Med. 2014 Feb;20(2):116-27. doi: 10.1016/j.molmed.2013.10.007. Epub 2013 Dec 25. Trends Mol Med. 2014. PMID: 24374162 Review.
Structural dynamics associated with intermediate formation in an archetypal conformational disease.
Nyon MP, Segu L, Cabrita LD, Lévy GR, Kirkpatrick J, Roussel BD, Patschull AO, Barrett TE, Ekeowa UI, Kerr R, Waudby CA, Kalsheker N, Hill M, Thalassinos K, Lomas DA, Christodoulou J, Gooptu B. Nyon MP, et al. Among authors: lomas da. Structure. 2012 Mar 7;20(3):504-12. doi: 10.1016/j.str.2012.01.012. Structure. 2012. PMID: 22405009 Free PMC article.
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. Gooptu B, et al. Among authors: lomas da. J Mol Biol. 2009 Apr 10;387(4):857-68. doi: 10.1016/j.jmb.2009.01.069. Epub 2009 Feb 14. J Mol Biol. 2009. PMID: 19232354 Free PMC article.
pH-dependent stability of neuroserpin is mediated by histidines 119 and 138; implications for the control of beta-sheet A and polymerization.
Belorgey D, Hägglöf P, Onda M, Lomas DA. Belorgey D, et al. Among authors: lomas da. Protein Sci. 2010 Feb;19(2):220-8. doi: 10.1002/pro.299. Protein Sci. 2010. PMID: 19953505 Free PMC article.
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