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    Number of items: 22.

    Article

    Lomas, D.A. and Hurst, J.R. and Gooptu, Bibekbrata (2016) Update on alpha-1 antitrypsin deficiency: new therapies. Journal of Hepatology 65 (2), pp. 413-424. ISSN 0168-8278.

    Haq, I. and Irving, J. and Saleh, A.D. and Dron, L. and Regan-Mochrie, G.L. and Motamedi-Shad, N. and Hurst, J.R. and Gooptu, Bibek and Lomas, D.A. (2016) Deficiency mutations of α1-antitrypsin differentially affect folding, function and polymerization. American Journal of Respiratory Cell and Molecular Biology 54 (1), pp. 71-80. ISSN 1044-1549.

    Moriconi, C. and Ordoñez, A. and Lupo, G. and Gooptu, Bibek and Irving, J.A. and Noto, R. and Martorana, V. and Manno, M. and Timpano, V. and Guadagno, N.A. and Dalton, L. and Marciniak, S.J. and Lomas, D.A. and Miranda, E. (2015) Interactions between N-linked glycosylation and polymerisation of neuroserpin within the endoplasmic reticulum. FEBS Journal 282 (23), pp. 4565-4579. ISSN 1742-464X.

    Nyon, M.P. and Prentice, T. and Day, J. and Kirkpatrick, J. and Sivalingam, G.N. and Lévy, Géraldine R. and Haq, I. and Irving, J. and Lomas, D.A. and Christodoulou, John and Gooptu, Bibek and Thalassinos, Konstantinos (2015) An integrative approach combining ion mobility mass spectrometry, X-ray crystallography and NMR spectroscopy to study the conformational dynamics of α1-antitrypsin upon ligand binding. Protein Science 24 (8), pp. 1301-1312. ISSN 0961-8368.

    Nyon, M.P. and Prentice, T. and Day, J. and Kirkpatrick, J. and Sivalingam, G.N. and Levy, G. and Haq, I. and Irving, J.A. and Lomas, D.A. and Christodoulou, John and Gooptu, Bibek and Thalassinos, Konstantinos (2015) 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. Protein Science 24 (8), pp. 1301-1312. ISSN 0961-8368.

    Tan, L. and Perez, J. and Mela, M. and Miranda, E. and Burling, K.A. and Rouhani, F.N. and DeMeo, D.L. and Haq, I. and Irving, J.A. and Ordóñez, A. and Dickens, J.A. and Brantly, M. and Marciniak, S.J. and Alexander, G.J. and Gooptu, Bibek and Lomas, D.A. (2015) Characterising the association of latency with α1-antitrypsin polymerisation using a novel monoclonal antibody. The International Journal of Biochemistry & Cell Biology 58 , pp. 81-91. ISSN 1357-2725.

    Gooptu, Bibek and Dickens, J.A. and Lomas, D.A. (2014) The molecular and cellular pathology of α1-antitrypsin deficiency. Trends in Molecular Medicine 20 (2), pp. 116-127. ISSN 1471-4914.

    Haq, I. and Irving, J.A. and Faull, S.V. and Dickens, J.A. and Ordóñez, A. and Belorgey, D. and Gooptu, Bibek and Lomas, D.A. (2013) Reactive centre loop mutants of α-1-antitrypsin reveal position-specific effects on intermediate formation along the polymerization pathway. Bioscience Reports 33 (3), pp. 499-511. ISSN 0144-8463.

    Nyon, Mun Peak and Segu, Lakshmi and Cabrita, L.D. and Lévy, Géraldine R. and Kirkpatrick, J. and Roussel, B.D. and Patschull, Anathe O.M. and Barrett, Tracey E. and Ekeowa, U.I. and Kerr, R. and Waudby, Christopher A. and Kalsheker, N. and Hill, M. and Thalassinos, Konstantinos and Lomas, D.A. and Christodoulou, John and Gooptu, Bibek (2012) Structural dynamics associated with intermediate formation in an archetypal conformational disease. Structure 20 (3), pp. 504-512. ISSN 0969-2126.

    Patschull, Anathe O.M. and Segu, Lakshmi and Nyon, Mun Peak and Lomas, D.A. and Nobeli, Irene and Barrett, Tracey E. and Gooptu, Bibek (2011) Therapeutic target-site variability in α1-antitrypsin characterized at high resolution. Acta Crystallographica Section F Structural Biology and Crystallization Communications 67 (12), pp. 1492-1497. ISSN 1744-3091.

    Ekeowa, U.I. and Freeke, J. and Miranda, E. and Gooptu, Bibek and Bush, M.F. and Pérez, J. and Teckman, J. and Robinson, C.V. and Lomas, D.A. (2010) Defining the mechanism of polymerization in the serpinopathies. Proceedings of the National Academy of Sciences of the United States of America 107 (40), pp. 17146-17151. ISSN 0027-8424.

    Miranda, E. and Pérez, J. and Ekeowa, U.I. and Hadzic, N. and Kalsheker, N. and Gooptu, Bibek and Portmann, B. and Belorgey, D. and Hill, M. and Chambers, S. and Teckman, J. and Alexander, G.J. and Marciniak, S.J. and Lomas, D.A. (2010) A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with α1-antitrypsin deficiency. Hepatology 52 (3), pp. 1078-1088. ISSN 0270-9139.

    Gooptu, Bibek and Ekeowa, U.I. and Lomas, D.A. (2009) Mechanisms of emphysema in 1-antitrypsin deficiency: molecular and cellular insights. European Respiratory Journal 34 (2), pp. 475-488. ISSN 0903-1936.

    Gooptu, Bibek and Lomas, D.A. (2009) Conformational pathology of the serpins: themes, variations, and therapeutic strategies. Annual Review of Biochemistry 78 (1), pp. 147-176. ISSN 0066-4154.

    Ekeowa, U.I. and Gooptu, Bibek and Belorgey, D. and Hägglöf, P. and Karlsson‑Li, S. and Miranda, E. and Pérez, J. and MacLeod, I. and Kroger, H. and Marciniak, S.J. and Crowther, D.C. and Lomas, D.A. (2009) α1-Antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies. Clinical Science 116 (12), pp. 837-850. ISSN 0143-5221.

    Gooptu, Bibek and Miranda, E. and Nobeli, Irene and Mallya, M. and Purkiss, Andrew G. and Leigh Brown, S.C. and Summers, C. and Phillips, R.L. and Lomas, D.A. and Barrett, Tracey E. (2009) Crystallographic and cellular characterisation of two mechanisms stabilising the native fold of α1-Antitrypsin: implications for disease and drug design. Journal of Molecular Biology 387 (4), pp. 857-868. ISSN 0022-2836.

    Gooptu, Bibek and Lomas, D.A. (2008) Polymers and inflammation: disease mechanisms of the serpinopathies. The Journal of Experimental Medicine 205 (7), pp. 1529-1534. ISSN 0022-1007.

    Mallya, M. and Phillips, R.L. and Saldanha, S.A. and Gooptu, Bibek and Leigh Brown, S.C. and Termine, D.J. and Shirvani, A.M. and Wu, Y. and Sifers, R.N. and Abagyan, R. and Lomas, D.A. (2007) Small molecules block the polymerization of Z α1-Antitrypsin and increase the clearance of intracellular aggregates. Journal of Medicinal Chemistry 50 (22), pp. 5357-5363. ISSN 0022-2623.

    Crowther, D.C. and Serpell, L.C. and Dafforn, T.R. and Gooptu, Bibek and Lomas, D.A. (2003) Nucleation of α1-Antichymotrypsin polymerization. Biochemistry 42 (8), pp. 2355-2363. ISSN 0006-2960.

    Gooptu, Bibek and Hazes, B. and Chang, W.-S.W. and Dafforn, T.R. and Carrell, R.W. and Read, R.J. and Lomas, D.A. (2000) Inactive conformation of the serpin alpha 1-antichymotrypsin indicates two-stage insertion of the reactive loop: implications for inhibitory function and conformational disease. Proceedings of the National Academy of Sciences of the United States of America 97 (1), pp. 67-72. ISSN 0027-8424.

    Davis, R.L. and Shrimpton, A.E. and Holohan, P.D. and Bradshaw, C. and Feiglin, D. and Collins, G.H. and Sonderegger, P. and Kinter, J. and Becker, L.M. and Lacbawan, F. and Krasnewich, D. and Muenke, M. and Lawrence, D.A. and Yerby, M.S. and Shaw, C.-M. and Gooptu, Bibek and Elliott, P.R. and Finch, J.T. and Carrell, R.W. and Lomas, D.A. (1999) Familial dementia caused by polymerization of mutant neuroserpin. Nature 401 , pp. 376-379. ISSN 0028-0836.

    Book Section

    Irving, J.A. and Ekeowa, U.I. and Belorgey, D. and Haq, I. and Gooptu, Bibek and Miranda, E. and Pérez, J. and Roussel, B.D. and Ordóñez, A. and Dalton, L.E. and Thomas, S.E. and Marciniak, S.J. and Parfrey, H. and Chilvers, E.R. and Teckman, J.H. and Alam, S. and Mahadeva, R. and Rashid, S.T. and Vallier, L. and Lomas, D.A. (2011) The serpinopathies: studying serpin polymerization in vivo. In: Whisstock, J.C. and Bird, P. I. (eds.) Serpin Structure and Evolution. Methods In Enzymology 501. New York, U.S.: Elsevier, pp. 421-466. ISBN 9780123859501.

    This list was generated on Wed Sep 22 05:01:06 2021 BST.