Bibek Gooptu: Publications

Management of Idiopathic Pulmonary Fibrosis
DAD Bruce-Hickman, H Garthwaite, M Heightman, B Gooptu
Book Chapter in “Clinical Handbook of Interstitial Lung Diseases” 2017 – in press
Eds. M Thillai, D Moller, K Meyer; Taylor & Francis

Alpha1-antitrypsin: structure and dynamics in health, disease and drug development
A Jagger, JA Irving ST Rashid, DA Lomas, B Gooptu
Book Chapter in “Alpha-1 Antitrypsin Deficiency” 2017 – in press
Eds. N Kalsheker and RA Stockley; Elsevier

A multicentre evaluation of multidisciplinary team meeting agreement on diagnosis in diffuse parenchymal lung disease: a case cohort study
SLF Walsh, AU Wells, SR Desai, V Poletti, S Piciucchi, A Dubini, H Nunes, D Valeyre, P Brillet, M Kambouchner, A Morais, JM Pereira, CP Souto Moura, JC Grutters, DA van den Heuvel, HW van Es, MF van Oosterhout, CA Seldenrijk, E Bendstrup, F Rasmussen, LB Madsen, B Gooptu, S Pomplun, H Taniguchi, J Fukuoka, T Johkoh, AG Nicholson, C Sayer, L Edmunds, J Jacob, MA Kokosi, JL Myers, KR Flaherty, D Hansell
Lancet Respiratory Medicine – 4(7):557-65. doi: 10.1016/S2213-2600(16)30033-9. 2016

Update on alpha-1 antitrypsin deficiency: new therapies
DA Lomas, JR Hurst, B Gooptu
Journal of Hepatology – 65(2):413-24, 2016

Aberrant disulphide bonding contributes to the ER retention of alpha1-antitrypsin deficiency variants
R Ronzoni, R Berardelli, D Medicina, R Sitia, B Gooptu, AM Fra
Human Molecular Genetics, 25(4):642-50, doi: 10.1093/hmg/ddv501, 2016

Deficiency mutations of α1-antitrypsin. Effects on folding, function and polymerisation
I Haq, JA Irving, AD Saleh, L Dron, JR Hurst, G Regan-Mochrie, N Motamedi-Shad, B Gooptu§, DA Lomas§
(§ joint senior/corresponding author)
Am J Resp Cell Mol Biol, 54:71-80. doi: 10.1165/rcmb.2015-0154OC, PMID: 26091018, 2016

Interactions between N-linked glycosylation and polymerisation of neuroserpin within the endoplasmic reticulum
E Miranda, C Moriconi, A Ordóñez, B Gooptu, JA Irving, V Timpano, L Dalton, SJ Marciniak, DA Lomas
FEBS J, 282(23):4565-79 – doi: 10.1111/febs.13517, PMID: 26367528, 2015

Controversies in COPD: α1-antitrypsin deficiency
D Bruce-Hickman, CM Greene, B Gooptu
European Respiratory Monograph, 2015

Misfolding and polymerisation of 1-antitrypsin – conformational pathology and therapeutic targeting
B Gooptu, DA Lomas
Book Chapter in “Alpha-1 Antitrypsin: Role in Health and Disease” 2015
Eds. A Wanner and RA Sandhaus; Humana Press

Native ion mobility mass spectrometry, crystallographic and NMR spectroscopic characterisation of α1-antitrypsin conformational dynamics relevant to disease and therapy
MP Nyon, T Prentice, J Day, J Kirkpatrick, G Sivalingam, G Levy, I Haq, JA Irving, DA Lomas, J Christodoulou, B Gooptu§, Konstantinos Thalassinos§
(§ joint senior/corresponding author)
Protein Science, 2015, 24:1301-1312

Associations of latency with 1-antitrypsin polymerisation
L Tan, J Perez, M Mela, E Miranda, KA Burling, FN Rouhani, DL DeMeo, I Haq, JA Irving, A Ordóñez, JA Dickens, M Brantly, SJ Marciniak, GJM Alexander, B Gooptu§, DA Lomas§
(§ joint senior/corresponding author)
International Journal of Biochemistry and Cell Biology, 2015 58:81-91

Tacrolimus in IIM-associated ILD: defining roles and responders
P Gordon and B Gooptu
Rheumatology, 2015 54(1):3-4

Therapeutic targeting of misfolding and conformational change in α1-antitrypsin deficiency
MP Nyon and B Gooptu
Future Medicinal Chemistry, 2014 6:1047-1065

The molecular and cellular pathology of α₁-antitrypsin deficiency
B Gooptu, JA Dickens, DA Lomas
Trends in Molecular Medicine, 2014 20:116-127

Reactive centre loop mutants of α-1-antitrypsin reveal position-specific effects on intermediate formation along the polymerization pathway
I Haq, JA Irving, SV Faull, JA Dickens, A Ordóñez, D Belorgey, B Gooptu, DA Lomas
Bioscience Reports, 2013 33:e00046c

Structural dynamics associated with intermediate formation in an archetypal conformational disease
MP Nyon, L Segu, LD Cabrita, G Lévy, J Kirkpatrick, BD Roussel, AOM Patschull, R Kerr, UI Ekeowa, TE Barrett, N Kalsheker, M Hill, K Thalassinos, DA Lomas, J Christodoulou, B Gooptu
Structure 2012, 20:504-12

Three new alpha1-antitrypsin deficiency variants help to define a C-terminal region regulating conformational change and polymerization
AM Fra, B Gooptu, I Ferrarotti, R Scabini, E Miranda, R Ronzoni, F Benini, L Corda, D Medicina, M Luisetti, L Schiaffonati
PLoS ONE 2012: e38405. doi:10.1371/journal.pone.0038405

In silico assessment of potential druggable pockets on the surface of α1-antitrypsin conformers
AOM Patschull, B Gooptu, P Ashford, T Daviter, I Nobeli
PLoS ONE 2012 7: e36612. doi:10.1371/journal.pone.0036612

1H, 15N and 13C backbone resonance assignments of the archetypal serpin α1-antitrypsin
MP Nyon, J Kirkpatrick, LD Cabrita, J Christodoulou, B Gooptu
Biomol NMR Assign. 2012 6:153-6. doi: 10.1007/s12104-011-9345-y

Therapeutic target site variability in α1-antitrypsin characterized at high resolution
AOM Patschull, L Segu, MP Nyon, DA Lomas, I Nobeli, TE Barrett, B Gooptu
Acta Crystallographica F 2011, 67:1492-1497

The structural biology of α1-antitrypsin and the serpinopathies
B Gooptu and DA Lomas
Book chapter in “Methods in Protein Biochemistry”, 2011 Ed. H Tschesche, De Gruyter, Berlin, New York

Targeting serpins in high-throughput and structure-based drug design
YP Chang, R Mahadeva, AOM Patschull, I Nobeli, AR McKay, K Thalassinos, JA Irving, I Haq, MP Nyon, J Christodoulou, UI Ekeowa, A Ordóñez, E Miranda,
B Gooptu
Methods in Enzymology 2011, 501:139-75

The serpinopathies: Studying serpin polymerisation in vivo
JA Irving, UI Ekeowa, D Belorgey, I Haq, B Gooptu, E Miranda, J Pérez, BD Roussel, A Ordóñez, LE Dalton, SE Thomas, SJ Marciniak, H Parfrey, ER Chilvers, JH Teckman, S Alam, R Mahadeva, ST Rashid, L Vallier, DA Lomas
Methods in Enzymology 2011, 501:421-66

Defining the mechanism of polymerization in the serpinopathies
UI Ekeowa, J Freeke, E Miranda, B Gooptu, M Bush, J Pérez, J Teckman, CV Robinson, DA Lomas
Proceedings of the National Academy of Sciences (USA) 2010, 107:17146-17151

A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with α1-antitrypsin deficiency
E Miranda, J Pérez, UI Ekeowa, N Hadzic, N Kalsheker, B Gooptu, B Portmann, D Belorgey, M Hill, S Chambers, J Teckman, GJ Alexander, SJ Marciniak, DA Lomas
Hepatology 2010, 52: 1078-1088

Alpha1-antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies
UI Ekeowa, B Gooptu, D Belorgey, P Hägglöf, S Karlsson-Li, E Miranda, J Pérez, I MacLeod, H Kroger, SJ Marciniak, DC Crowther, DA Lomas
Clinical Science 2009, 116: 837-850

Mechanisms of emphysema in α1-antitrypsin deficiency: molecular and cellular insights
B Gooptu, UI Ekeowa, DA Lomas
European Respiratory Journal 2009, 34: 475-488

Conformational pathology of the serpins – themes, variations and therapeutic strategies
B Gooptu and DA Lomas
Annual Review of Biochemistry 2009, 78:147-176

Crystallographic and cellular characterisation of two mechanisms stabilising the native fold of alpha1-antitrypsin: implications for disease and drug design
B Gooptu, E Miranda, I Nobeli, M Mallya, AG Purkiss, SC Leigh Brown, C Summers, RL Phillips, DA Lomas, TE Barrett
Journal of Molecular Biology 2009, 387: 865-868

Polymers and inflammation: disease mechanisms of the serpinopathies
B Gooptu and DA Lomas
Journal of Experimental Medicine 2008, 205: 1529-1534

Small molecules block the polymerization of Z α1-antitrypsin and increase the clearance of intracellular aggregates
M Mallya, RL Phillips, SA Saldanha, B Gooptu, SC Leigh Brown, DJ Termine, AM Shirvani, Y Wu, RN Sifers, R Abagyan, DA Lomas
Journal of Medicinal Chemistry 2007, 50: 5357-5363

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
B Gooptu, L Ward, SO Ansari, CD Eraut, D Law, AG Davison
Emergency Medicine Journal 2006, 23:636-638

Nucleation of 1-antichymotrypsin polymerization
DC Crowther, LC Serpell, TR Dafforn, B Gooptu, DA Lomas
Biochemistry 2003, 42:2355-6

Inactive conformation of the serpin 1-antichymotrypsin indicates two-stage insertion of the reactive loop; implications for the inhibitory function and conformational disease
B Gooptu, B Hazes, W-S W Chang, TR Dafforn, RW Carrell, RJ Read, DA Lomas
Proceedings of the National Academy of Sciences (USA) 2000, 97;67-72

Familial dementia caused by polymerisation of mutant neuroserpin
RL Davis, AE Shrimpton, P Holohan, C Bradshaw, D Feiglin, P Sonderegger, J Kinter, LM Becker, F Lacbawan, D Krasnewich, M Muenke, DA Lawrence, MS Yerby, C-M Shaw, B Gooptu, PR Elliott, JT Finch, RW Carrell, DA Lomas
Nature 1999, 401:376-378

Conformational changes and disease – serpins, prions and Alzheimer’s
RW Carrell and B Gooptu
Current Opinion in Structural Biology 1998, 8:799-809

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