Prof Stephen Bottomley - Researcher Profile

Biography

Professor Stephen Bottomley is Senior Research Fellow of the NHMRC and he leads a committed team of researchers who's goal is to understand and prevent a range of devestating diseases caused by protein misfolding.  

Qualifications

PHD

Institution: University of Southampton

Year awarded: 1994

B.SC. (HONS)

Institution: University of East Anglia

Year awarded: 1990

Publications

Book Chapters

Kaiserman, D., Hitchen, C.R., Levina, V., Bottomley, S.P., Bird, P.I., 2011, Intracellular production of recombinant serpins in yeast, in Methods in Enzymology, Volume 501: Serpin Structure and Evolution, eds James C Whisstock and Phillip I Bird, Academic Press, USA, pp. 1-12.

Horvath, A.J., Lu, B.G., Pike, R.N., Bottomley, S.P., 2011, Methods to measure the kinetics of protease inhibition by serpins, in Methods in Enzymology, Volume 501: Serpin Structure and Evolution, eds James C Whisstock and Phillip I Bird, Academic Press, USA, pp. 223-235.

Phan, J., Yamout, N., Schmidberger, J., Bottomley, S., Buckle, A., 2011, Refolding Your Protein with a Little Help from REFOLD, in Protein Folding, Misfolding, and Disease, eds AF Hill, KJ Barnham, SP Bottomley and R Cappai, Springer Science+Business Media, UK, pp. 45-57.

Pearce, M.C., Pike, R.N., Lesk, A.M., Bottomley, S.P., 2007, Serpin Conformations, in Molecular and Cellular Aspects of the Serpinopathies and Disorders in Serpin Activity, eds G.A. Silverman & D.A. Lomas, World Scientific, Singapore, pp. 35-66.

Zhang, Q., Law, R.H.P., Buckle, A.M., Cabrita, L., McGowan, S., Irving, J.A., Faux, N.G., Lesk, A.M., Bottomley, S.P., Whisstock, J., 2007, Serpins in Prokaryotes, in Molecular and Cellular Aspects of the Serpinopathies and Disorders in Serpin Activity, eds G.A. Silverman & D.A. Lomas, World Scientific, Singapore, pp. 131-162.

Chow, M.K., Ellisdon, A.M., Cabrita, L., Bottomley, S.P., 2006, Purification of polyglutamine proteins, in Methods in Enzymology Volume 413: Amyloid, Prions and Other Protein Aggregates, Part C, eds Indu Kheterpal and Ronald Wetzel, Academic Press, USA, pp. 1-19.

Cabrita, L.D., Bottomley, S.P., 2004, Protein expression and refolding - a practical guide to getting the most out of inclusion bodies, in Biotechnology Annual Review Volume 10, eds M Raafat El-Gewely, Elsevier B.V., Amsterdam The Netherlands, pp. 31-50.

Journal Articles

Knaupp, A.S., Keleher, S.B., Yang, L.L., Dai, W., Bottomley, S.P., Pearce, M.C., 2013, The Roles of Helix I and Strand 5A in the Folding, Function and Misfolding of alpha(1)-Antitrypsin, PLoS ONE [P], vol 8, issue 1, Public Library of Science, USA, pp. 1-8.

Kass, I., Knaupp, A., Bottomley, S.P., Buckle, A.M., 2012, Conformational properties of the disease-causing Z variant of a1-antitrypsin revealed by theory and experiment, Biophysical Journal [P], vol 102, issue 12, Cell Press, USA, pp. 2856-2865.

Samson, A.L., Knaupp, A.S., Sashindranath, M., Borg, R.J., Au, A.E., Cops, E.J., Saunders, H.M., Cody, S.H., McLean, C., Nowell, C.J., Hughes, V.A., Bottomley, S.P., Medcalf, R.L., 2012, Nucleocytoplasmic coagulation: an injury-induced aggregation event that disulfide crosslinks proteins and facilitates their removal by plasmin, Cell Reports [E], vol 2, issue 4, Cell Press, USA, pp. 889-901.

Singh, Y., Sharpe, P.C., Hoang, H.N., Lucke, A.J., McDowall, A., Bottomley, S.P., Fairlie, D.P., 2011, Amyloid formation from an alpha-helix peptide bundle is seeded by 3(10)-helix aggregates, Chemistry-A European Journal [P], vol 17, issue 1, Wiley - VCH Verlag GmbH & Co. KGaA, Germany, pp. 151-160.

Ng, N., Pierce, M.J., Webb, G., Ratnikov, B., Wijeyewickrema, L.C., Duncan, R., Robertson, A.L., Bottomley, S.P., Boyd, S.E., Pike, R.N., 2011, Discovery of amino acid motifs for thrombin cleavage and validation using a model substrate, Biochemistry [P], vol 50, issue 48, American Chemical Society, USA, pp. 10499-10507.

Saunders, H., Gilis, D., Rooman, M., Dehouck, Y., Robertson, A., Bottomley, S., 2011, Flanking domain stability modulates the aggregation kinetics of a polyglutamine disease protein, Protein Science [P], vol 20, issue 10, Wiley-Blackwell, USA, pp. 1675-1681.

Buckle, A.M., Bate, M.A., Androulakis, S.G., Cinquanta, M., Basquin, J., Bonneau, F., Chatterjee, D.K., Cittaro, D., Graslund, S., Gruszka, A., Page, R., Suppmann, S., Wheeler, J.X., Agostini, D., Taussig, M., Taylor, C.F., Bottomley, S.P., Villaverde, A., de Marco, A., 2011, Recombinant protein quality evaluation: proposal for a minimal information standard, Standards in Genomic Sciences [E], vol 5, issue 2, Michigan State University * Genomic Standards Consortium, USA, pp. 195-197.

Knaupp, A.S., Bottomley, S.P., 2011, Structural change in beta-sheet A of Z alpha{1}-antitrypsin is responsible for accelerated polymerization and disease, Journal of Molecular Biology [P], vol 413, issue 4, Academic Press, UK, pp. 888-898.

Robertson, A., Bate, M., Buckle, A., Bottomley, S., 2011, The rate of polyQ-mediated aggregation is dramatically affected by the number and location of surrounding domains, Journal of Molecular Biology [P], vol 413, issue 4, Academic Press, UK, pp. 879-887.

Bottomley, S., 2011, The structural diversity in alpha{1}-antitrypsin misfolding, Embo Reports [P], vol 12, issue 10, Nature Publishing Group, UK, pp. 983-984.

Pearce, M.C., Powers, G.A., Feil, S.C., Hansen, G., Parker, M.W., Bottomley, S.P., 2010, Identification and characterization of a misfolded monomeric serpin formed at physiological temperature, Journal of Molecular Biology [P], vol 403, issue 3, Academic Press, UK, pp. 459-467.

Knaupp, A.S., Levina, V., Robertson, A.L., Pearce, M.C., Bottomley, S.P., 2010, Kinetic instability of the serpin Z alpha1-antitrypsin promotes aggregation, Journal of Molecular Biology [P], vol 396, issue 2, Academic Press, UK, pp. 375-383.

Robertson, A.L., Bate, M.A., Androulakis, S.G., Bottomley, S.P., Buckle, A.M., 2010, PolyQ: A database describing the sequence and domain context of polyglutamine repeats in proteins, Nucleic Acids Research [P], vol 39, issue Database issue, Oxford University Press, UK, pp. D272-D276.

Silverman, G.A., Whisstock, J.C., Bottomley, S.P., Huntington, J.A., Kaiserman, D., Luke, C.J., Pak, S.C., Reichhart, J.-., Bird, P.I., 2010, Serpins flex their muscle: I. Putting the clamps on proteolysis in diverse biological systems, Journal Of Biological Chemistry [P], vol 285, issue 32, American Society for Biochemistry and Molecular Biology Inc., USA, pp. 24299-24305.

Whisstock, J.C., Silverman, G.A., Bird, P.I., Bottomley, S.P., Kaiserman, D., Luke, C.J., Pak, S.C., Reichhart, J.-., Huntington, J.A., 2010, Serpins flex their muscle: II. Structural insights into target peptidase recognition, polymerization, and transport functions, Journal Of Biological Chemistry [P], vol 285, issue 32, American Society for Biochemistry and Molecular Biology Inc., USA, pp. 24307-24312.

Robertson, A.L., Headey, S.J., Saunders, H.M., Ecroyd, H., Scanlon, M., Carver, J.A., Bottomley, S.P., 2010, Small heat-shock proteins interact with a flanking domain to suppress polyglutamine aggregation, Proceedings Of The National Academy Of Sciences Of The United States Of America [P], vol 107, issue 23, National Academy of Sciences, USA, pp. 10424-10429.

Bottomley, S.P., 2010, The folding pathway of {alpha}1-Antitrypsin: Avoiding the unavoidable, Proceedings of the American Thoracic Society [P], vol 7, issue 6, American Thoracic Society, USA, pp. 404-407.

Kennan, R.M., Wong, W., Dhungyel, O., Han, X., Wong, D.M., Parker, D., Rosado, C.J., Law, R.H., McGowan, S., Reeve, S.B., Levina, V., Powers, G.A., Pike, R.N., Bottomley, S.P., Smith, A.I., Marsh, I., Whittington, R.J., Whisstock, J., Porter, C.J., Rood, J.I., 2010, The subtilisin-like protease AprV2 is required for virulence and uses a novel disulphide-tethered exosite to bind substrates, Plos Pathogens [P], vol 6, issue 11, Public Library of Science, USA, pp. 1-12.

Robertson, A.L., Bottomley, S.P., 2010, Towards the treatment of polyglutamine diseases: The modulatory role of protein context, Current Medicinal Chemistry [P], vol 17, issue 27, Bentham Science Publishers Ltd, The Netherlands, pp. 3058-3068.

Beddoe, T.C., Chen, Z., Clements, C.S., Ely, L.K., Bushell, S.R., Vivian, J.P., Kjer-Nielsen, L., Pang, S.S., Dunstone, M.A., Liu, Y.C., Macdonald, W.A., Perugini, M.A., Wilce, M.C.J., Burrows, S.R., Purcell, A.W., Tiganis, T., Bottomley, S.P., McCluskey, J., Rossjohn, J., 2009, Antigen ligation triggers a conformational change within the constant domain of the alphabeta T cell receptor, Immunity [P], vol 30, issue 6, Cell Press, USA, pp. 777-788.

Ong, P.C., Golding, S.J., Pearce, M.C., Irving, J.A., Grigoryev, S.A., Pike, D., Langendorf, C.G., Bashtannyk-Puhalovich, T.A., Bottomley, S.P., Whisstock, J.C., Pike, R.N., McGowan, S., 2009, Conformational change in the chromatin remodelling protein MENT, PL o S One, vol 4, issue 3 (e4727), Public Library of Science, USA, pp. 1-5.

Levina, V., Dai, W., Knaupp, A.S., Kaiserman, D., Pearce, M.C., Cabrita, L.D., Bird, P.I., Bottomley, S.P., 2009, Expression, purification and characterization of recombinant Z alpha1-Antitrypsin-The most common cause of alpha1-Antitrypsin deficiency, Protein Expression And Purification [P], vol 68, issue 2, Academic Press, USA, pp. 226-232.

Niclis, J.C., Trounson, A.O., Dottori, M., Ellisdon, A.M., Bottomley, S.P., Verlinsky, Y., Cram, D.S., 2009, Human embryonic stem cell models of Huntington disease, Reproductive Biomedicine Online [P], vol 19, issue 1, Reproductive Healthcare Ltd, UK, pp. 106-113.

Saunders, H.M., Bottomley, S.P., 2009, Multi-domain misfolding: understanding the aggregation pathway of polyglutamine proteins, Protein Engineering, Design and Selection [P], vol 22, issue 8, Oxford University Press, UK, pp. 447-451.

Verstak, B., Nagpal, K., Bottomley, S.P., Golenbock, D.T., Hertzog, P.J., Mansell, A.S., 2009, MyD88 adapter-like (Mal)/TIRAP interaction with TRAF6 is critical for TLR2- and TLR4-mediated NF-kappaB proinflammatory responses, Journal Of Biological Chemistry [P], vol 284, issue 36, The American Society for Biochemistry and Molecular Biology, Inc, United States, pp. 24192-24203.

Knaupp, A.S., Bottomley, S.P., 2009, Serpin polymerization and its role in disease - The molecular basis of alpha1-antitrypsin deficiency, IUBMB Life, vol 61, issue 1, Wiley-Blackwell Publishing Ltd, United Kingdom, Switzerland, pp. 1-5.

Paxman, J.J., Borg, N., Horne, H.J., Thompson, P., Chin, K.Y., Sharma, P., Simpson, J.S., Wielens, J., Piek, S., Kahler, C.M., Sakellaris, H., Pearce, M.C., Bottomley, S.P., Rossjohn, J., Scanlon, M., 2009, The Structure of the Bacterial Oxidoreductase Enzyme DsbA in Complex with a Peptide Reveals a Basis for Substrate Specificity in the Catalytic Cycle of DsbA Enzymes, Journal Of Biological Chemistry [P], vol 284, issue 26, American Society for Biochemistry and Molecular Biology, Inc., USA, pp. 17835-17845.

Zhang, Q., Law, R.H.P., Bottomley, S.P., Whisstock, J., Buckle, A.M., 2008, A structural basis for Loop C-sheet polymerization in serpins, Journal of Molecular Biology, vol 376, issue 5, Academic Press, United Kingdom, pp. 1348-1359.

Fulton, Z.J., McAlister, A.D., Wilce, M.C., Brammananth, R., Zaker-Tabrizi, L., Perugini, M.A., Bottomley, S.P., Coppel, R.L., Crellin, P., Rossjohn, J., Beddoe, T.C., 2008, Crystal structure of a UDP-glucose-specific glycosyltransferase from a Mycobacterium species, Journal of Biological Chemistry, vol 283, issue 41, American Society for Biochemistry and Molecular Biology, Inc., United States, pp. 27881-27890.

Smith, D.P., Tew, D., Hill, A.F., Bottomley, S.P., Masters, C.L., Barnham, K.J., Cappai, R., 2008, Formation of a high affinity lipid-binding intermediate during the early aggregation phase of alpha-synuclein, Biochemistry, vol 47, issue 5, American Chemical Society, United States, pp. 1425-1434.

Shaw, G.S., Marlatt, N.M., Ferguson, P.L., Barber, K.R., Bottomley, S.P., 2008, Identification of a dimeric intermediate in the unfolding pathway for the calcium-binding protein S100B, Journal of Molecular Biology, vol 382, issue 4, Academic Press, United Kingdom, pp. 1075-1088.

Pearce, M.C., Morton, C.J., Feil, S.C., Hansen, G., Adams, J.J., Parker, M.W., Bottomley, S.P., 2008, Preventing serpin aggregation: The molecular mechanism of citrate action upon antitrypsin unfolding, Protein Science, vol 17, issue 12, Wiley-Blackwell Publishing, Inc., United States, pp. 2127-2133.

Tew, D.J., Bottomley, S.P., Smith, D.P., Ciccotosto, G., Babon, J., Hinds, M.G., Masters, C.L., Cappai, R., Barnham, K.J., 2008, Stabilization of Neurotoxic Soluble beta-Sheet-Rich Conformations of the Alzheimer's Disease Amyloid-beta Peptide, Biophysical Journal, vol 94, Biophysical Society, USA, pp. 2752-2766.

Vivian, J., Scoullar, J., Robertson, A.L., Bottomley, S.P., Horne, H.J., Chin, K.Y., Wielens, J., Thompson, P., Velkov, T., Piek, S., Byres, E., Beddoe, T.C., Wilce, M.C., Kahler, C.M., Rossjohn, J., Scanlon, M., 2008, Structural and biochemical characterization of the oxidoreductase NmDsbA3 from Neisseria meningitidis, The Journal of Biological Chemistry, vol 283, issue 47, American Society for Biochemistry and Molecular Biology Inc., United States, pp. 32452-32461.

Whisstock, J., Bottomley, S.P., 2008, Structural biology: Serpins' mystery solved, Nature, vol 455, issue 7217, Nature Publishing Group, United Kingdom, pp. 1189-1190.

Robertson, A.L., Horne, H.J., Ellisdon, A.M., Thomas, B., Scanlon, M., Bottomley, S.P., 2008, The structural impact of a polyglutamine tract is location-dependent, Biophysical Journal, vol 95, issue 12, Biophysical Society, United States, pp. 5922-5930.

Rosado, C.J., Buckle, A.M., Law, R.H.P., Butcher, R.E., Kan, W., Bird, C.H., Ung, K., Browne, K.A., Baran, K., Bashtannyk-Puhalovich, T.A., Faux, N.G., Wong, W., Porter, C.J., Pike, R.N., Ellisdon, A.M., Pearce, M.C., Bottomley, S.P., Emsley, J., Smith, A.I., Rossjohn, J., Hartland, E.L., Voskoboinik, I., Trapani, J.A., Bird, P.I., Dunstone, M.A., Whisstock, J., 2007, A common fold mediates vertebrate defense and bacterial attack, Science, vol 317, issue 5844, American Association of Advancement in Science, Washington, DC, USA, pp. 1548-1551.

Cabrita, L., Irving, J.A., Pearce, M.C., Whisstock, J., Bottomley, S.P., 2007, Aeropin from the extremophile pyrobaculum aerophilum bypasses the serpin misfolding trap, Journal of Biological Chemistry, vol 282, issue 37, American Society of Biochemistry Molecular Biology Inc, Bethesda MD USA, pp. 26802-26809.

Ong, P.C., McGowan, S., Pearce, M.C., Irving, J.A., Kan, W., Grigoryev, S.A., Turk, B., Silverman, G.A., Brix, K., Bottomley, S.P., Whisstock, J., Pike, R.N., 2007, DNA accelerates the inhibition of human cathepsin V by serpins, Journal of Biological Chemistry, vol 282, issue 51, American Society of Biochemistry & Molecular Biology Inc, Bethesda MD USA, pp. 36980-36986.

Cabrita, L., Gilis, D., Robertson, A.L., Dehouck, Y., Rooman, M., Bottomley, S.P., 2007, Enhancing the stability and solubility of TEV protease using in silico design, Protein Science, vol 16, issue 11, Cold Spring Harbor Lab Press Publications Dept, Woodbury NY USA, pp. 2360-2367.

Myers, G.S., Parker, D., Al-Hasani, K., Kennan, R.M., Seemann, T., Ren, Q., Badger, J.H., Selengut, J.D., DeBoy, R.T., Tettelin, H., Boyce, J.D., McCarl, V., Han, X.Y., Nelson, W.C., Madupu, R., Mohamoud, Y., Holley, T., Fedorova, N., Bottomley, S.P., Whittington, R.J., Adler, B., Songer, J., Rood, J.I., Paulsen, I.T., 2007, Genome sequence and identification of candidate vaccine antigens from the animal pathogen Dichelobacter nodosus, Nature Biotechnology, vol 25, issue 5, Nature Publishing Group, New York NY USA, pp. 569-575.

Al-Hasani, K., Boyce, J.D., McCarl, V., Bottomley, S.P., Wilkie, I.W., Adler, B., 2007, Identification of novel immunogens in Pasteurella multocida, Microbial Cell Factories, vol 6, issue 3, BioMed Central Ltd, London England UK, pp. 1-5.

Ellisdon, A.M., Pearce, M.C., Bottomley, S.P., 2007, Mechanisms of ataxin-3 misfolding and fibril formation: kinetic analysis of a disease-associated polyglutamine protein, Journal of Molecular Biology, vol 368, issue 2, Academic Press Ltd Elsevier Science Ltd, London England UK, pp. 595-605.

Powers, G.A., Pham, C.L., Pearce, M.C., Howlett, G.J., Bottomley, S.P., 2007, Serpin acceleration of amyloid fibril formation: a role for accessory proteins, Journal of Molecular Biology, vol 366, issue 2, Academic Press Ltd, Elsevier Science Ltd, London England, pp. 666-676.

Zhang, Q., Buckle, A.M., Law, R.H.P., Pearce, M.C., Cabrita, L., Lloyd, G., Irving, J.A., Smith, A.I., Ruzyla, K., Rossjohn, J., Bottomley, S.P., Whisstock, J., 2007, The N terminus of the serpin, tengpin, functions to trap the metastable native state, EMBO Reports, vol 8, issue 7, Nature Publishing Group, UK, pp. 658-663.

Pearce, M.C., Cabrita, L., Ellisdon, A.M., Bottomley, S.P., 2007, The loss of tryptophan 194 in antichymotrypsin lowers the kinetic barrier to misfolding, FEBS Journal, vol 274, issue 14, Blackwell Publishing, Oxford England UK, pp. 3622-3632.

Cabrita, L.D., Dai, W., Bottomley, S.P., 2006, A family of E. coli expression vectors for laboratory scale and high throughput soluble protein production, BMC Biotechnology, vol 6, issue 12, Biomed Central Ltd, London UK, pp. 1-8.

Whisstock, J., Bottomley, S.P., 2006, Molecular gymnastics: serpin structure, folding and misfolding, Current Opinion in Structural Biology, vol 16, issue 6, Current Biology Ltd, London UK, pp. 761-768.

Chow, M.K., Amin, A.A., Fulton, K.F., Whisstock, J.C., Buckle, A.M., Bottomley, S.P., 2006, REFOLD: an analytical database of protein refolding methods, Protein Expression and Purification, vol 46, issue 1, Academic Press Inc Elsevier Science, San Diego USA, pp. 166-171.

Chow, M.K.M., Amin, A.A., Fulton, K.F., Fernando, T., Kamau, L., Batty, C., Louca, M., Ho, S., Whisstock, J.C., Bottomley, S.P., Buckle, A.M., 2006, The REFOLD database: a tool for the optimization of protein expression and refolding., Nucleic Acids Research, vol 34, issue D-Base Issue, Oxford University Press, Oxford UK, pp. D207-D212.

Ellisdon, A.M., Thomas, B., Bottomley, S.P., 2006, The two-stage pathway of ataxin-3 Fibrillogenesis involves a polyglutamine-independent step, The Journal of Biological Chemistry, vol 281, issue 25, The American Society for Biochemistry and Molecular Biology Inc., Bethesda USA, pp. 16888-16896.

Bushell, S.R., Bottomley, S.P., Rossjohn, J., Beddoe, T.C., 2006, Tracking the unfolding pathway of a multirepeat protein via tryptophan scanning: Evidence of localized instability in the mitochondrial import receptor Tom70, The Journal of Biological Chemistry, vol 281, issue 34, American Society for Biochemistry and Molecular Biology, Inc, Bethesda USA, pp. 24345-24350.

McGowan, S., Buckle, A.M., Irving, J.A., Ong, P.C., Bashtannyk-Puhalovich, T.A., Kan, W., Henderson, K., Bulynko, Y.A., Popova, E.Y., Smith, A.I., Bottomley, S.P., Rossjohn, J., Grigoryev, S.A., Pike, R.N., Whisstock, J.C., 2006, X-ray crystal structure of MENT: evidence for functional loop-sheet polymers in chromatin condensation, The EMBO Journal, vol 25, issue 13, Nature Publishing Group, New York USA, pp. 3144-3155.

Devlin, G.L., Bottomley, S.P., 2005, A protein family under 'stress' - serpin stability, folding and misfolding, Frontiers in Bioscience, vol 10, issue 1, Frontiers in Bioscience Inc., Manhasset USA, pp. 288-299.

Bushell, S.R., Beddoe, T.C., Perugini, M.A., Bottomley, S.P., Rossjohn, J., 2005, A structural and functional characterization of Tom70: a receptor of the mitochondrial import machinery, Febs Journal, vol 272, Blackwell Publishing, Oxford England, p. 365.

Ely, L.K., Green, K.J., Beddoe, T.C., Clements, C.S., Miles, J.J., Bottomley, S.P., Zernich, D., Kjer-Nielsen, L., Purcell, A.W., McCluskey, J., Rossjohn, J., Burrows, S.R., 2005, Antagonism of antiviral and allogeneic activity of a human public ctl clonotype by a single altered peptide ligand: Implications for allograft rejection, Journal of Immunology, vol 174, issue 9, American Association of Immunologists, Bethesda USA, pp. 5593-5601.

Cappai, R., Leck, S., Tew, D.J., Williamson, N.A., Smith, D.P., Galatis, D., Sharples, R.A., Curtain, C.C., Ali, F.E., Cherny, R.A., Culvenor, J.G., Bottomley, S.P., Masters, C.L., Barnham, K.J., Hill, A.F., 2005, Dopamine promotes alpha-synuclein aggregation into SDS-resistant soluble oligomers via a distinct folding pathway, The FASEB Journal, vol 19, issue 10, Federation of American Societies for Experimental Biology, Bethesda USA, pp. 1377-1379.

Faux, N.G., Bottomley, S.P., Lesk, A.M., Irving, J.A., Morrison, J.R., Garcia De La Banda, M.J., Whisstock, J.C., 2005, Functional insights from the distribution and role of homopeptide repeat-containing proteins, Genome Research, vol 15, issue 4, Cold Spring Harbor Lab Press, Woodbury USA, pp. 537-551.

Fulton, K.F., Devlin, G.L., Jodun, R.A., Silvestri, L., Bottomley, S.P., Ferscht, A.R., Buckle, A.M., 2005, PFD: a database for the investigation of protein folding kinetics and stability, Nucleic Acids Research, vol 33, issue suppl 1, Oxford University Press, Oxford UK, pp. D279-D283.

Whisstock, J., Bottomley, S.P., Bird, P.I., Pike, R.N., Coughlin, P.B., 2005, Serpins 2005 - fun between the beta-sheets : Meeting report based upon presentations made at the 4th International Symposium on Serpin Structure, Function and Biology (Cairns, Australia), FEBS Journal, vol 272, issue 19, Blackwell Publishing, Oxford UK, pp. 4868-4873.

Fulton, K.F., Buckle, A.M., Cabrita, L.D., Irving, J.A., Butcher, R.E., Smith, A.I., Reeve, S.B., Lesk, A.M., Bottomley, S.P., Rossjohn, J., Whisstock, J.C., 2005, The high resolution crystal structure of a native thermostable serpin reveals the complex mechanism underpinning the stressed to relaxed transition, Journal of Biological Chemistry, vol 280, issue 9, American Society for Biochemistry and Molecular Biology, Inc., Bethesda USA, pp. 8435-8442.

Law, R.H.P., Irving, J.A., Buckle, A.M., Ruzyla, K., Buzza, M.S., Bashtannyk-Puhalovich, T.A., Beddoe, T.C., Nguyen, K., Worrall, M.D., Bottomley, S.P., Bird, P.I., Rossjohn, J., Whisstock, J.C., 2005, The high resolution crystal structure of the human tumor suppressor maspin reveals a novel conformational switch in the G-helix, Journal of Biological Chemistry, vol 280, issue 23, American Society for Biochemistry and Molecular Biology, Inc., Bethesda USA, pp. 22356-22364.

Buckle, A.M., Devlin, G.L., Jodun, R.A., Fulton, K.F., Faux, N.G., Whisstock, J.C., Bottomley, S.P., 2005, The matrix refolded, Nature Methods, vol 2, issue 1, Nature Publishing Group, London UK, p. 3.

Horvath, A.J., Irving, J.A., Rossjohn, J., Law, R.H.P., Bottomley, S.P., Quinsey, N.S., Pike, R.N., Coughlin, P.B., Whisstock, J., 2005, The murine orthologue of human antichymotrypsin: a structural paradigm for CLADE A3 serpins, Journal of Biological Chemistry, vol 280, issue 52, American Society for Biochemistry and Molecular Biology, Inc., Bethesda USA, pp. 43168-43178.

Beddoe, T.C., Bushell, S.R., Perugini, M.A., Lithgow, T., Mulhern, T.D., Bottomley, S.P., Rossjohn, J., 2004, A biophysical analysis of the tetratricopeptide repeat-rich mitochondrial import receptor, Tom70, reveals an elongated monomer that is inherently flexible, unstable, and unfolds via a multistate pathway, Journal of Biological Chemistry, vol 279, issue 45, The American Society for Biochemistry and Molecular Biology, Inc., Bethesda USA, pp. 46448-46454.

Quinsey, N.S., Greedy, A.L., Bottomley, S.P., Whisstock, J.C., Pike, R.N., 2004, Antithrombin: in control of coagulation, The International Journal of Biochemistry & Cell Biology, vol 36, issue 3, Pergamon-Elsevier Science Ltd, Kidlington England, pp. 386-389.

Chow, M.K., Paulson, H.P., Bottomley, S.P., 2004, Destabilization of a non-pathological variant of ataxin-3 results in fibrillogenesis via a partially folded intermediate: A model for misfolding in polyglutamine disease, Journal of Molecular Biology, vol 335, issue 1, Academic Press Ltd Elsevier Science Ltd, London England, pp. 333-341.

Cabrita, L.D., Dai, W., Bottomley, S.P., 2004, Different conformational changes within the F-helix occur during serpin folding, polymerization, and proteinase inhibition, Biochemistry, vol 43, issue 30, American Chemical Society, Washington USA, pp. 9834-9839.

Pearce, M.C., Cabrita, L.D., Rubin, H., Gore, M.G., Bottomley, S.P., 2004, Identification of residual structure within denatured antichymotrypsin: implications for serpin folding and misfolding, Biochemical and Biophysical Research Communications, vol 324, issue 2, Academic Press Inc. Elsevier Science, San Diego USA, pp. 729-735.

Zernich, D., Purcell, A.W., Macdonald, W.A., Kjer-Nielsen, L., Ely, L.K., Laham, N., Crockford, T., Mifsud, N.A., Bharadwaj, M., Chang, L., Tait, B.D., Holdsworth, R., Brooks, A.G., Bottomley, S.P., Beddoe, T.C., Peh, C.A., Rossjohn, J., McCluskey, J., 2004, Natural HLA class I polymorphism controls the pathway of antigen presentation and susceptibility to viral evasion, The Journal of Experimental Medicine, vol 200, issue 1, Rockerfeller University Press, New York USA, pp. 13-24.

Dafforn, T.R., Pike, R.N., Bottomley, S.P., 2004, Physical characterization of serpin conformations, Methods, vol 32, issue 2, Academic Press Inc. Elsevier Science, San Diego USA, pp. 150-158.

Chow, M.K., Ellisdon, A.M., Cabrita, L.D., Bottomley, S.P., 2004, Polyglutamine expansion in ataxin-3 does not affect protein stability, The Journal of Biological Chemistry, vol 279, issue 46, The American Society for Biochemistry and Molecular Biology, Inc., Bethesda USA, pp. 47643-47651.

Bird, P.I., Pak, S.C., Worrall, D.M., Bottomley, S.P., 2004, Production of recombinant serpins in Escherichia coli, Methods, vol 32, issue 2, Academic Press Inc. Elsevier Science, San Diego USA, pp. 169-176.

Chow, M.K., Lomas, D., Bottomley, S.P., 2004, Promiscuous beta-strand interactions and the conformational diseases, Current Medicinal Chemistry, vol 11, issue 4, Bentham Science Publishers Ltd, Hilversum The Netherlands, pp. 491-499.

Chow, M.K., Mackay, J.P., Whisstock, J.C., Scanlon, M.J., Bottomley, S.P., 2004, Structural and functional analysis of the Josephin domain of the polyglutamine protein ataxin-3, Biochemical and Biophysical Research Communications, vol 322, issue 3, Academic Press Inc. Elsevier Science, San Diego USA, pp. 387-394.

Benning, L.N., Whisstock, J.C., Sun, J., Bird, P.I., Bottomley, S.P., 2004, The human serpin proteinase inhibitor-9 self-associates at physiological temperatures, Protein Science, vol 13, issue 7, Cold Spring Harbor Laboratory Press, Woodbury USA, pp. 1859-1864.

Ellisdon, A.M., Bottomley, S.P., 2004, The role of protein misfolding in the pathogenesis of human diseases, IUBMB Life, vol 56, issue 3, Taylor & Francis Inc., Philadelphia USA, pp. 119-123.

Webb, A.I., Borg, N.A., Dunstone, M.A., Kjer-Nielsen, L., Beddoe, T.C., McCluskey, J., Carbone, F.R., Bottomley, S.P., Aguilar, M.I., Purcell, A.W., Rossjohn, J., 2004, The structure of H-2Kb and Kbm8 complexed to a herpes simplex virus determinant: evidence for a conformational switch that governs T cell repertoire selection and viral resistance, The Journal of Immunology, vol 173, issue 1, American Association of Immunologists, Bethesda USA, pp. 402-409.

Ludeman, J.P., Pike, R.N., Bromfield, K.M., Duggan, P.J., Cianci, J., Le Bonniec, B., Whisstock, J.C., Bottomley, S.P., 2003, Determination of the P'1, P'2 and P'3 subsite-specificity of factor Xa, International Journal of Biochemistry & Cell Biology, vol 35, issue 2, Pergamon-Elsevier Science Ltd, Oxford England, pp. 221-225.

Cabrita, L.D., Bottomley, S.P., 2003, How do proteins avoid becoming too stable? Biophysical studies into metastable proteins, European BIophysics Journal, vol 33, issue 2, Springer-Verlag, New York USA, pp. 83-88.

Gilis, D., McLennan, H.R., Dehouck, Y., Cabrita, L.D., Rooman, M., Bottomley, S.P., 2003, In vitro and in silico design of 1-antitrypsin mutants with different conformational stabilities, Journal of Molecular Biology, vol 325, issue 3, Academic Press Ltd Elsevier Science Ltd, London England, pp. 581-589.

Quinsey, N.S., Fitton, H.L., Coughlin, P.B., Whisstock, J.C., Dafforn, T.R., Carrell, R.W., Bottomley, S.P., Pike, R.N., 2003, Introduction of a mutation in the shutter region of antithrombin (Phe77 Leu) increases affinity for heparin and decreases thermal stability, Biochemistry, vol 42, issue 34, American Chemical Society, Washington USA, pp. 10169-10173.

Irving, J.A., Cabrita, L.D., Rossjohn, J., Pike, R.N., Bottomley, S.P., Whisstock, J.C., 2003, The 1.5 crystal structure of a prokaryote serpin: controlling conformational change in a heated environment, Structure, vol 11, issue 4, Cell Press, Cambridge USA, pp. 387-397.

Devlin, G.L., Carver, J.A., Bottomley, S.P., 2003, The selective inhibition of sepin aggregation by the molecular chaperone, -crystallin indicates a nucleation-dependent specificity, Journal of Biological Chemistry, vol 278, issue 49, American Society for Biochemistry and Molecular Biology, Inc., Bethesda USA, pp. 48644-48650.

Devlin, G., Chow, M.K., Howlett, G.J., Bottomley, S., 2002, Acid denaturation of 1-antitrypsin: Characterization of a novel mechanism of serpin polymerization, Journal of Molecular Biology, vol 324, issue 4, Academic Press Ltd Elsevier Science, London UK, pp. 859-870.

De Niese, M.R., Chinni, C., Pike, R.N., Bottomley, S., Mackie, E.J., 2002, Dissection of protease-activated receptor-1-dependent and -independent responses to thrombin in skeletal myoblasts, Experimental Cell Research, vol 274, issue 1, Academic Press Inc Elsevier Science, San Diego CA USA, pp. 149-156.

Irving, J., Pike, R.N., Dai, W., Bromme, D., Worrall, M.D., Silverman, G.A., Coetzer, T.H., Dennison, C., Bottomley, S., Whisstock, J.C., 2002, Evidence that serpin architecture intrinsically supports papain-like cystein protease inhibition: Engineering 1- abutrypsin to inhibit acathepsin proteases, Biochemistry, vol 41, issue 15, American Chemical Society, Washington DC USA, pp. 4998-5004.

Irving, J.A., Shushanov, S., Pike, R.N., Popova, E.Y., Bromme, D., Coetzer, T.H., Bottomley, S., Boulynko, I.A., Grigoreyev, S.A., Whisstock, J.C., 2002, Inhibitory activity of a heterchromatin-associated serpin (MENT) against papain like cystein proteinases affects chromatin structure and blocks cell proliferation, The Journal of Biological Chemistry, vol 277, issue 15, American Society Biochemistry Molecular Biology Inc, Bethesda MD USA, pp. 13192-13201.

Quinsey, N.S., Whisstock, J.C., Le Bonniec, B., Louvain, V., Bottomley, S.P., Pike, R.N., 2002, Molecular determinants of the mechanisms underlying acceleration of the interaction between antithrombin and factor Xa by heparin pentasaccharide, The Journal of Biological Chemistry, vol 277, issue 18, American Society of Biochemistry and Molecular Biology, Bethesda MS USA, pp. 15971-15978.

Cabrita, L., Whisstock, J.C., Bottomley, S., 2002, Probing the role of the F-Helix in serpin stability through a single tryptophan substitution, Biochemistry, vol 41, issue 14, American Chemical Society, Washington DC USA, pp. 4575-4581.

Pike, R.N., Bottomley, S., Irving, J., Bird, P.I., Whisstock, J.C., 2002, Serpins: finely balanced conformational traps, IUBMB Life, vol 54, issue 1, Taylor & Francis, Philadelphia PA USA, pp. 1-7.

Tew, D.J., Bottomley, S., 2001, Intrinsic flurescence changes and rapid kinetics of proteinase deformation during serpin inhibition, FEBS Letters, vol 494, Elsevier Science BV, Amsterdam Netherlands, pp. 30-33.

Chow, M.K., Devlin, G., Bottomley, S., 2001, Osmolytes as modulators of conformational changes in serpins, Biological Chemistry, vol 382, Walter De Gruyter & Co, Berlin Germany, pp. 1593-1599.

Devlin, G., Parfrey, H., Tew, D.J., Lomas, D., Bottomley, S., 2001, Prevention of polymerization of M and Z a1-antitrypsin (a1-AT) with trimethylamine N-Oxide . Implications for the treatment of a1-AT deficiency, American Journal of Respiratory Cell and Molecular Biology, vol 24, American Thoracic Society, New York NY USA, pp. 727-732.

Tew, D.J., Bottomley, S., 2001, Probing the equilibrium denaturation of the serpin a1-antitrypsin with single tryptophan mutants; evidence for structure in the urea unfolded state, Journal of Molecular Biology, vol 313, Academic Press Ltd, London England, pp. 1161-1169.

Ludeman, J.P., Whisstock, J.C., Hopkins, P., Le Bonniec, B., Bottomley, S., 2001, Structure of a serpin-enzyme complex probed by cysteine substitutions and fluorescence spectroscopy, Biophysical Journal, vol 80, Biophysical Society, Bethesda MD USA, pp. 491-497.

Bottomley, S., Lawrenson, I.D., Tew, D.J., Dai, W., Whisstock, J.C., Pike, R.N., 2001, The role of strand 1 of the C b-sheet in the structure and function of a1- antitrypsin, Protein Science, vol 10, Cold Spring Harbor Laboratory Press, Plainview New York NY USA, pp. 2518-2524.

Dunstone, M.A., Dai, W., Whisstock, J.C., Rossjohn, J., Pike, R.N., Feil, S.C., Le Bonniec, B., Parker, M.W., Bottomley, S.P., 2000, Cleaved antitrypsin polymers at atominc resolution, Protein Science, vol 9, Cambridge University Press, New York NY USA, pp. 417-420.

Pearce, M.C., Rubin, H., Bottomley, S.P., 2000, Conformational Change and Intermediates in the Unfolding of 1-Antichymotrypsin, The Journal of Biological Chemistry, vol 275, issue 37, The American Society for Biochemistry & Molecular Biology, Inc, Bethesda MD USA, pp. 28513-28518.

Chinni, C., De Niese, M.R., Jenkins, A.L., Pike, R.N., Bottomley, S.P., Mackie, E.J., 2000, Protease-activated receptor-2 mediates proliferative responses in skeletal myoblasts, Journal of Cell Science, vol 113, Company of Biologists Ltd, Cambridge UK, pp. 4427-4433.

Bottomley, S.P., Tew, D.J., 2000, The citrate ion increases the conformational stability of alpha1-antitrypsin, Biochimica Et Biophysica Acta, vol 1481, Elsevier Science BV, Amsterdam Netherlands, pp. 11-17.

Whisstock, J.C., Romero, S., Gurung, R., Nandurkar, H., Ooms, L.M., Bottomley, S.P., Mitchell, C.A., 2000, The inositol polyphosphate 5-phosphatases and the apurinic/apyrimidinic base excision repair endonucleases share a common mechanism for catalysis, Journal of Biological Chemistry, vol 275 issue 47, Amer Soc for Biochem & Molecular Biology, Bethesda USA, pp. 37055-37061.

James, E.L., Whisstock, J.C., Gore, M.G., Bottomley, S.P., 1999, Probing the unfolding pathway of alpha1-antitrypsin, Journal of Biological Chemistry, vol 274, no 14, The American Soc. for Biochem. and Mol. Biol., USA, pp. 9482-9488.

Whisstock, J.C., Irving, J., Bottomley, S.P., Pike, R.N., Lesk, A.M., 1999, Serpins in the "Caenorhabditis elegans" genome, Proteins-Structure Function and Genetics, vol 36, Wiley-Liss, Inc., URL:http://www.sanger.ac.uk/Projects/C_elegans/wo/, pp. 31-41.

Scott, F.L., Hirst, C., Sun, J., Bird, C., Bottomley, S.P., Bird, P.I., 1999, The intracellular serpin proteinase inhibitor 6 is expressed in monocytes and granulocytes and is a potent inhibitor of the azurophilic granule protease, Cathepsin G, Blood, vol 93 issue 6, American Society of Haematology, Washington DC USA, pp. 2089-2097.

Scott, F.L., Hirst, C., Sun, J., Bird, C., Bottomley, S.P., Bird, P.I., 1999, The intracellular serpin proteinase inhibitor 6 is expressed in monocytes and granulocytes and is a potent inhibitor of the azurophilic granule proterase, Cathepsin G, Blood, vol 93, no 6, The American Society of Hematology, USA, pp. 2089-2097.

Chinni, C., De Niese, M.R., Tew, D.J., Jenkins, A.L., Bottomley, S.P., Mackie, E.J., 1999, Thrombin, a survival factor for cultured myoblasts, Journal of Biological Chemistry, vol 274, no 14, The American Soc. for Biochem. and Mol. Biol., www.jbc.org, pp. 9169-9174.

Bottomley, S.P., Stone, S.R., 1998, Protein engineering of chimeric Serpins: an investigation into effects of the serpin scaffold and reactive centre loop length, Protein Engineering, vol 11 no 12, Oxford University Press, Oxford UK, pp. 1243-1247.

James, E.L., Bottomley, S.P., 1998, The mechanism of a1-antitrypsin polymerization probed by fluorescence spectroscopy, Archives of Biochemistry and Biophysics, vol 356, no 2, Academic Press, Orlando FL USA, pp. 296-300.

Bottomley, S.P., Hopkins, P., Whisstock, J.C., 1998, a1 - antitrypsin polymerisation can occur by both Loop A and C sheet mechanisms, Biochemical and Biophysical Research Communications, vol 251 no 1, Academic Press, Orlando FL USA, pp. 1-5.

Sun, J., Bottomley, S., Kumar, S., Bird, P.I., 1997, Recombinant caspase - 3 expressed in Pichia pastoris is fully activated and kinetically indistinguishable from the native enzyme, Biochemical and Biophysical Research Communications, vol 238, Academic Press, Orlando FL USA, pp. 920-924.

Sun, J., Bottomley, S.P., Kumar, S., Bird, P.I., 1997, Reconbinant Caspase-3 Expressed in "Pichia pastoris" is Fully Activiated and Kinetically Indistinguishable for the Native Enzyme, Biochemical and Biophysical Research Communications, vol 238, Academic Press, USA, pp. 920-924.

Bottomley, S., Chang, W., 1997, The effects of reactive centre loop length upon serpin polymerisation, Biochemical and Biophysical Research Communications, vol 241, Academic Press, Orlando FL USA, pp. 264-269.

Conference Proceedings

Bottomley, S.P., James, E.L., Whisstock, J.C., 1999, Biophysical and biochemical analysis of the alpha1-antitrypsin folding and misfolding pathways, Proceedings, 24th Annual Lorne Conference on Protein and Structure Function, Lorne Vic, Lorne Protein Workshop, Inc., Melbourne Vic Australia, p. B22.

Smith, S.R., Whisstock, J.C., Lawrenson, I.D., Hopkins, P., Ludeman, J., Bottomley, S.P., Pike, R.N., 1999, Engineering a novel factor Xa inhibitor, Proceedings, 24th Annual Conference on Protein Structure and Function, Lorne Vic 7-11 February 1999, Lorne Protein Workshop, Inc., Melbourne Vic Australia, p. B12.

Lawrenson, I.D., Whisstock, J.C., Pike, R.N., Bottomley, S.P., 1999, The importance of strand 1C in the inhibitory mechanism of alpha1-antitrypsin, Proceedings, 24th Annual Conference on Protein Structure and Function, Lorne Vic 7-11 February 1999, Lorne Protein Workshop, Inc., Melbourne Vic Australia, p. C38.

James, E.L., Whisstock, J.C., Bottomley, S.P., 1998, The unfolding pathway of a1 - antitrypsin probed by florecence spectroscopy, Proceedings, 23rd Annual Lorne Conference on Protein Structure and Function, Lorne VIC 8-12 February 1998, Lorne Protein Workshop, Inc., Melbourne Vic Australia, p. 28.

Postgraduate Research Supervisions

Current Supervision

Program of Study:

(DOCTORATE BY RESEARCH).

Thesis Title:

Analysing the conformational changes associated with polyglutamine protein aggregation.

Supervisors:

Bottomley, S (Main).

Program of Study:

(DOCTORATE BY RESEARCH).

Thesis Title:

Biophysical characterisation of the josphin domain of ataxin-3.

Supervisors:

Bottomley, S (Main).

Program of Study:

(DOCTORATE BY RESEARCH).

Thesis Title:

Producing a Danio rerio (zebrafish) model of Z antitrypsin.

Supervisors:

Bottomley, S (Main), Bird, P (Associate), Verkade, H (Associate).

Program of Study:

(DOCTORATE BY RESEARCH).

Thesis Title:

Serpin folding and misfolding.

Supervisors:

Bottomley, S (Main).

Program of Study:

(DOCTORATE BY RESEARCH).

Thesis Title:

The structure and specificacy of deconjugating enzymes USP5 and USP13.

Supervisors:

Bottomley, S (Main).

Completed Supervision

Student:

Benning, L.

Program of Study:

The intracellular serpins PI-6 and PI-9: an analysis of conformational change. (PHD) 2007.

Supervisors:

Bottomley, S (Main), Bird, P (Joint).

Student:

Cabrita, L.

Program of Study:

STRUCTURAL AND BIOPHYSICAL INVESTIGATIONS INTO THE ROLE OF METASTABILITY IN SERPIN FOLDING, MISFOLDING AND INHIBITION. (PHD) 2004.

Supervisors:

Bottomley, S (Main), Aguilar, M (Associate).

Student:

Chow, M.

Program of Study:

MECHANISMS OF FOLDING AND MISFOLDING BY ATAXIN-3, A POLYGLUTAMINE PROTEIN. (PHD) 2004.

Supervisors:

Bottomley, S (Main), Aguilar, M (Associate).

Student:

Devlin, G.

Program of Study:

THE MECHANISMS OF SERPIN MISFOLDING AND ITS IBHIBITION. (PHD) 2003.

Supervisors:

Bottomley, S (Main), Aguilar, M (Associate).

Student:

Di Stefano, P.

Program of Study:

Polyglutamine repeat protein fibrillogenesis and its inhibition. 2012.

Supervisors:

Bottomley, S (Main).

Student:

Ellisdon, A.

Program of Study:

Mechanisms of ataxin-3 misfolding and fibril formation. (PHD) 2006.

Supervisors:

Bottomley, S (Main).

Student:

James, E.

Program of Study:

A SPECTROSCOPIC COMPARISON OF THE GUANIDINE HYDROCHLORIDE MEDIATED UNFOLDING AND REFOLDING PATHWAYS OF NATIVE AND CLEAVED ALPHA-1-ANTITRYPSIN. (Masters) 2000.

Supervisors:

Bottomley, S (Main), Aguilar, M (Associate).

Student:

Knaupp, A.

Program of Study:

Biophysical characterisation of the pathological Z variant of alpha-1-antitrypsin. (PHD) 2012.

Supervisors:

Bottomley, S (Main).

Student:

Lupton, C.

Program of Study:

Protein Misfolding. 2010.

Supervisors:

Bottomley, S (Main).

Student:

Mccarl, V.

Program of Study:

Structural and functional studies of nitrilases from Arabidopsis thaliana. (PHD) 2007.

Supervisors:

Langford, S (Main), Bottomley, S (Associate).

Student:

Pearce, M.

Program of Study:

A STUDY INTO THE FOLDING AND MISFOLDING MECHANISMS OF ANTICHYMOTRYPSIN. (PHD) 2004.

Supervisors:

Bottomley, S (Main), Aguilar, M (Associate).

Student:

Powers, G.

Program of Study:

Antichymotrypsin misfolding and its role in disease. (PHD) 2009.

Supervisors:

Bottomley, S (Joint-Co).

Student:

Robertson, A.

Program of Study:

An investigation into polyglutamine aggregation mechanisms. (PHD) 2009.

Supervisors:

Bottomley, S (Main).

Student:

Saunders, H.

Program of Study:

Investigation of the multi-domain aggregation mechanism of ataxin-3. (PHD) 2010.

Supervisors:

Bottomley, S (Main).

Student:

Tew, D.

Program of Study:

a1-ANTITRYPSIN: A SPECTROSCOPIC STUDY OF THE FOLDING AND INHIBITORY PATHWAYS OF A SERPIN. (PHD) 2002.

Supervisors:

Bottomley, S (Main).

Student:

Thomas, B.

Program of Study:

Aggregation of the polyglutamine protein ataxin-3. (PHD) 2009.

Supervisors:

Bottomley, S (Main).