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    Article

    Ronzoni, R. and Berardelli, R. and Medicina, D. and Sitia, R. and Gooptu, Bibek and Fra, A.M. (2016) Aberrant disulphide bonding contributes to the ER retention of alpha1-antitrypsin deficiency variants. Human Molecular Genetics 25 (4), pp. 642-650. ISSN 0964-6906.

    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.

    Chambers, J.E. and Dalton, L.E. and Subramanian, D.E. and Gooptu, Bibek and Balan, A. and Park, S.-M. and Holden, S. and Marciniak, S.J. (2015) Spontaneous pneumothorax can be associated with TGFBR2 mutation. European Respiratory Jurnal 46 (6), pp. 1832-1835. ISSN 0903-1936.

    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.

    Gordon, P. and Gooptu, Bibek (2015) Tacrolimus in idiopathic inflammatory myopathy-associated interstitial lung disease: defining roles and responders. Rheumatology 54 (1), pp. 3-4. ISSN 1462-0324.

    Nyon, Mun Peak and Gooptu, Bibek (2014) Therapeutic targeting of misfolding and conformational change in α1-antitrypsin deficiency. Future Medicinal Chemistry 6 (9), pp. 1047-1065. ISSN 1756-8919.

    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 Kirkpatrick, J. and Cabrita, L.D. and Christodoulou, John and Gooptu, Bibek (2012) 1H, 15N and 13C backbone resonance assignments of the archetypal serpin α1-antitrypsin. Biomolecular NMR Assignments 6 (2), pp. 153-156. ISSN 1874-2718.

    Patschull, Anathe O.M. and Gooptu, Bibek and Ashford, Paul and Daviter, Tina and Nobeli, Irene (2012) In silico assessment of potential druggable pockets on the surface of α1-Antitrypsin conformers. PLoS One 7 (5), ISSN 1932-6203.

    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.

    Gasset, M. and Fra, A.M. and Gooptu, Bibek and Ferrarotti, I. and Miranda, E. and Scabini, R. and Ronzoni, R. and Benini, F. and Corda, L. and Medicina, D. and Luisetti, M. and Schiaffonati, L. (2012) Three new Alpha1-Antitrypsin deficiency variants help to define a C-Terminal region regulating conformational change and polymerization. PLoS One 7 (6), ISSN 1932-6203.

    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.

    Gooptu, Bibek and Ward, L. and Ansari, S.O. and Eraut, C.D. and Law, D. and Davison, A.G. (2006) Oxygen alert cards and controlled oxygen: preventing emergency admissions at risk of hypercapnic acidosis receiving high inspired oxygen concentrations in ambulances and A&E departments. Emergency Medicine Journal 23 (8), pp. 636-638. ISSN 1472-0205.

    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.

    Carrell, R.W. and Gooptu, Bibek (1998) Conformational changes and disease — serpins, prions and Alzheimer's. Current Opinion in Structural Biology 8 (6), pp. 799-809. ISSN 0959-440X.

    Book Section

    Chang, Y.-P. and Mahadeva, R. and Patschull, A.O.M. and Nobeli, Irene and Ekeowa, U.I. and McKay, A.R. and Thalassinos, Konstantinos and Irving, J.A. and Haq, I. and Nyon, Mun Peak and Christodoulou, John and Ordóñez, A. and Miranda, E. and Gooptu, Bibek (2011) Targeting serpins in high-throughput and structure-based drug design. In: Whisstock, J.C. and Bird, P.I. (eds.) Serpin Structure and Evolution. Methods In Enzymology 501. New York, U.S.: Elsevier, pp. 139-175. ISBN 9780123859501.

    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.

    Gooptu, Bibek and Lomas, David A. (2011) The structural biology of α1-antitrypsin deficiency and the serpinopathies. In: Tschesche, H. (ed.) Methods in Protein Biochemistry. Berlin, Germany: DeGruyter, pp. 325-344. ISBN 9783110252361.

    This list was generated on Fri Jan 15 05:16:12 2021 GMT.