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Life and bladder cancer: protocol for a longitudinal and cross-sectional patient-reported outcomes study of Yorkshire (UK) patients.

Mason SJ, Downing A, Wright P, Bottomley SE, Winterbottom A, Glaser AW, Catto JWF.

BMJ Open. 2019 Jun 17;9(6):e030850. doi: 10.1136/bmjopen-2019-030850.


Use of virus-like particles as a native membrane model to study the interaction of insulin with the insulin receptor.

Sabapathy T, Helmerhorst E, Bottomley S, Babaeff S, Munyard K, Newsholme P, Mamotte CD.

Biochim Biophys Acta Biomembr. 2019 Jun 1;1861(6):1204-1212. doi: 10.1016/j.bbamem.2019.03.021. Epub 2019 Apr 2.


Reduced-toxicity allogeneic hematopoietic stem cell transplantation in congenital sideroblastic anemia.

Kim MH, Shah S, Bottomley SS, Shah NC.

Clin Case Rep. 2018 Aug 1;6(9):1841-1844. doi: 10.1002/ccr3.1667. eCollection 2018 Sep.


The phenotypic spectrum of germline YARS2 variants: from isolated sideroblastic anemia to mitochondrial myopathy, lactic acidosis and sideroblastic anemia 2.

Riley LG, Heeney MM, Rudinger-Thirion J, Frugier M, Campagna DR, Zhou R, Hale GA, Hilliard LM, Kaplan JA, Kwiatkowski JL, Sieff CA, Steensma DP, Rennings AJ, Simons A, Schaap N, Roodenburg RJ, Kleefstra T, Arenillas L, Fita-Torró J, Ahmed R, Abboud M, Bechara E, Farah R, Tamminga RYJ, Bottomley SS, Sanchez M, Huls G, Swinkels DW, Christodoulou J, Fleming MD.

Haematologica. 2018 Dec;103(12):2008-2015. doi: 10.3324/haematol.2017.182659. Epub 2018 Jul 19.


Recurrent heteroplasmy for the MT-ATP6 p.Ser148Asn (m.8969G>A) mutation in patients with syndromic congenital sideroblastic anemia of variable clinical severity.

Berhe S, Heeney MM, Campagna DR, Thompson JF, White EJ, Ross T, Peake RWA, Hanrahan JD, Rodriguez V, Renaud DL, Patnaik MS, Chang E, Bottomley SS, Fleming MD.

Haematologica. 2018 Dec;103(12):e561-e563. doi: 10.3324/haematol.2018.199109. Epub 2018 Jul 13. No abstract available.


Health-related quality of life after treatment for bladder cancer in England.

Mason SJ, Downing A, Wright P, Hounsome L, Bottomley SE, Corner J, Richards M, Catto JW, Glaser AW.

Br J Cancer. 2018 May;118(11):1518-1528. doi: 10.1038/s41416-018-0084-z. Epub 2018 May 14.


Evaluating patient-reported outcome measures (PROMs) for bladder cancer: a systematic review using the COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) checklist.

Mason SJ, Catto JWF, Downing A, Bottomley SE, Glaser AW, Wright P.

BJU Int. 2018 Nov;122(5):760-773. doi: 10.1111/bju.14368. Epub 2018 Jun 8.


Probing the folding pathway of a consensus serpin using single tryptophan mutants.

Yang L, Irving JA, Dai W, Aguilar MI, Bottomley SP.

Sci Rep. 2018 Feb 1;8(1):2121. doi: 10.1038/s41598-018-19567-9.


Cost-effectiveness of routine imaging of suspected appendicitis.

D'Souza N, Marsden M, Bottomley S, Nagarajah N, Scutt F, Toh S.

Ann R Coll Surg Engl. 2018 Jan;100(1):47-51. doi: 10.1308/rcsann.2017.0132. Epub 2017 Oct 19.


The self-association of HMGB1 and its possible role in the binding to DNA and cell membrane receptors.

Anggayasti WL, Mancera RL, Bottomley S, Helmerhorst E.

FEBS Lett. 2017 Jan;591(2):282-294. doi: 10.1002/1873-3468.12545. Epub 2017 Jan 20. Review.


Physicochemical properties that control protein aggregation also determine whether a protein is retained or released from necrotic cells.

Samson AL, Ho B, Au AE, Schoenwaelder SM, Smyth MJ, Bottomley SP, Kleifeld O, Medcalf RL.

Open Biol. 2016 Nov;6(11). pii: 160098.


Altered RECQL5 expression in urothelial bladder carcinoma increases cellular proliferation and makes RECQL5 helicase activity a novel target for chemotherapy.

Patterson K, Arya L, Bottomley S, Morgan S, Cox A, Catto J, Bryant HE.

Oncotarget. 2016 Nov 15;7(46):76140-76150. doi: 10.18632/oncotarget.12683.


Smoothing a rugged protein folding landscape by sequence-based redesign.

Porebski BT, Keleher S, Hollins JJ, Nickson AA, Marijanovic EM, Borg NA, Costa MG, Pearce MA, Dai W, Zhu L, Irving JA, Hoke DE, Kass I, Whisstock JC, Bottomley SP, Webb GI, McGowan S, Buckle AM.

Sci Rep. 2016 Sep 26;6:33958. doi: 10.1038/srep33958.


A recurring mutation in the respiratory complex 1 protein NDUFB11 is responsible for a novel form of X-linked sideroblastic anemia.

Lichtenstein DA, Crispin AW, Sendamarai AK, Campagna DR, Schmitz-Abe K, Sousa CM, Kafina MD, Schmidt PJ, Niemeyer CM, Porter J, May A, Patnaik MM, Heeney MM, Kimmelman A, Bottomley SS, Paw BH, Markianos K, Fleming MD.

Blood. 2016 Oct 13;128(15):1913-1917. Epub 2016 Aug 3.


The effect of physicochemical factors on the self-association of HMGB1: A surface plasmon resonance study.

Anggayasti WL, Mancera RL, Bottomley S, Helmerhorst E.

Biochim Biophys Acta. 2016 Nov;1864(11):1620-9. doi: 10.1016/j.bbapap.2016.07.008. Epub 2016 Jul 28.


Photodynamic killing of cancer cells by a Platinum(II) complex with cyclometallating ligand.

Doherty RE, Sazanovich IV, McKenzie LK, Stasheuski AS, Coyle R, Baggaley E, Bottomley S, Weinstein JA, Bryant HE.

Sci Rep. 2016 Mar 4;6:22668. doi: 10.1038/srep22668.


Optimization of surface plasmon resonance experiments: Case of high mobility group box 1 (HMGB1) interactions.

Anggayasti WL, Mancera RL, Bottomley S, Helmerhorst E.

Anal Biochem. 2016 Apr 15;499:43-50. doi: 10.1016/j.ab.2015.12.024. Epub 2016 Feb 8.


Congenital sideroblastic anemia due to mutations in the mitochondrial HSP70 homologue HSPA9.

Schmitz-Abe K, Ciesielski SJ, Schmidt PJ, Campagna DR, Rahimov F, Schilke BA, Cuijpers M, Rieneck K, Lausen B, Linenberger ML, Sendamarai AK, Guo C, Hofmann I, Newburger PE, Matthews D, Shimamura A, Snijders PJ, Towne MC, Niemeyer CM, Watson HG, Dziegiel MH, Heeney MM, May A, Bottomley SS, Swinkels DW, Markianos K, Craig EA, Fleming MD.

Blood. 2015 Dec 17;126(25):2734-8. doi: 10.1182/blood-2015-09-659854. Epub 2015 Oct 21.


Protein unfolding is essential for cleavage within the α-helix of a model protein substrate by the serine protease, thrombin.

Robertson AL, Headey SJ, Ng NM, Wijeyewickrema LC, Scanlon MJ, Pike RN, Bottomley SP.

Biochimie. 2016 Mar;122:227-34. doi: 10.1016/j.biochi.2015.09.021. Epub 2015 Sep 25.


Enhanced molecular mobility of ordinarily structured regions drives polyglutamine disease.

Lupton CJ, Steer DL, Wintrode PL, Bottomley SP, Hughes VA, Ellisdon AM.

J Biol Chem. 2015 Oct 2;290(40):24190-200. doi: 10.1074/jbc.M115.659532. Epub 2015 Aug 10.


Mutations in TRNT1 cause congenital sideroblastic anemia with immunodeficiency, fevers, and developmental delay (SIFD).

Chakraborty PK, Schmitz-Abe K, Kennedy EK, Mamady H, Naas T, Durie D, Campagna DR, Lau A, Sendamarai AK, Wiseman DH, May A, Jolles S, Connor P, Powell C, Heeney MM, Giardina PJ, Klaassen RJ, Kannengiesser C, Thuret I, Thompson AA, Marques L, Hughes S, Bonney DK, Bottomley SS, Wynn RF, Laxer RM, Minniti CP, Moppett J, Bordon V, Geraghty M, Joyce PB, Markianos K, Rudner AD, Holcik M, Fleming MD.

Blood. 2014 Oct 30;124(18):2867-71. doi: 10.1182/blood-2014-08-591370. Epub 2014 Sep 5.


The Z mutation alters the global structural dynamics of α1-antitrypsin.

Hughes VA, Meklemburg R, Bottomley SP, Wintrode PL.

PLoS One. 2014 Sep 2;9(9):e102617. doi: 10.1371/journal.pone.0102617. eCollection 2014.


Oxidation of an exposed methionine instigates the aggregation of glyceraldehyde-3-phosphate dehydrogenase.

Samson AL, Knaupp AS, Kass I, Kleifeld O, Marijanovic EM, Hughes VA, Lupton CJ, Buckle AM, Bottomley SP, Medcalf RL.

J Biol Chem. 2014 Sep 26;289(39):26922-36. doi: 10.1074/jbc.M114.570275. Epub 2014 Aug 1.


Sideroblastic anemia: diagnosis and management.

Bottomley SS, Fleming MD.

Hematol Oncol Clin North Am. 2014 Aug;28(4):653-70, v. doi: 10.1016/j.hoc.2014.04.008. Epub 2014 Jun 2. Review.


X-linked sideroblastic anemia due to ALAS2 intron 1 enhancer element GATA-binding site mutations.

Campagna DR, de Bie CI, Schmitz-Abe K, Sweeney M, Sendamarai AK, Schmidt PJ, Heeney MM, Yntema HG, Kannengiesser C, Grandchamp B, Niemeyer CM, Knoers NV, Swart S, Marron G, van Wijk R, Raymakers RA, May A, Markianos K, Bottomley SS, Swinkels DW, Fleming MD.

Am J Hematol. 2014 Mar;89(3):315-9. doi: 10.1002/ajh.23616. Epub 2013 Nov 20. Erratum in: Am J Hematol. 2014 Jun;89(6):670.


Conformational behavior and aggregation of ataxin-3 in SDS.

Saunders HM, Hughes VA, Cappai R, Bottomley SP.

PLoS One. 2013 Jul 22;8(7):e69416. doi: 10.1371/journal.pone.0069416. Print 2013.


A method for the incremental expansion of polyglutamine repeats in recombinant proteins.

Robertson AL, Bottomley SP.

Methods Mol Biol. 2013;1017:73-83. doi: 10.1007/978-1-62703-438-8_5.


A novel syndrome of congenital sideroblastic anemia, B-cell immunodeficiency, periodic fevers, and developmental delay (SIFD).

Wiseman DH, May A, Jolles S, Connor P, Powell C, Heeney MM, Giardina PJ, Klaassen RJ, Chakraborty P, Geraghty MT, Major-Cook N, Kannengiesser C, Thuret I, Thompson AA, Marques L, Hughes S, Bonney DK, Bottomley SS, Fleming MD, Wynn RF.

Blood. 2013 Jul 4;122(1):112-23. doi: 10.1182/blood-2012-08-439083. Epub 2013 Apr 3.


The roles of helix I and strand 5A in the folding, function and misfolding of α1-antitrypsin.

Knaupp AS, Keleher S, Yang L, Dai W, Bottomley SP, Pearce MC.

PLoS One. 2013;8(1):e54766. doi: 10.1371/journal.pone.0054766. Epub 2013 Jan 29.


Nucleocytoplasmic coagulation: an injury-induced aggregation event that disulfide crosslinks proteins and facilitates their removal by plasmin.

Samson AL, Knaupp AS, Sashindranath M, Borg RJ, Au AE, Cops EJ, Saunders HM, Cody SH, McLean CA, Nowell CJ, Hughes VA, Bottomley SP, Medcalf RL.

Cell Rep. 2012 Oct 25;2(4):889-901. doi: 10.1016/j.celrep.2012.08.026. Epub 2012 Oct 4.


Conformational properties of the disease-causing Z variant of α1-antitrypsin revealed by theory and experiment.

Kass I, Knaupp AS, Bottomley SP, Buckle AM.

Biophys J. 2012 Jun 20;102(12):2856-65. doi: 10.1016/j.bpj.2012.05.023. Epub 2012 Jun 19.


Predictive mutational bioinformatic analysis of variation in the skin and wool associated corneodesmosin (CDSN) gene in sheep.

Siva Subramaniam N, Morgan E, Bottomley S, Tay S, Gregg K, Lee CY, Wetherall J, Groth D.

Anim Sci J. 2012 May;83(5):386-93. doi: 10.1111/j.1740-0929.2011.00975.x. Epub 2011 Nov 16.


Molecular pathways to polyglutamine aggregation.

Robertson AL, Bottomley SP.

Adv Exp Med Biol. 2012;769:115-24. Review.


Recombinant protein quality evaluation: proposal for a minimal information standard.

Buckle AM, Bate MA, Androulakis S, Cinquanta M, Basquin J, Bonneau F, Chatterjee DK, Cittaro D, Gräslund S, Gruszka A, Page R, Suppmann S, Wheeler JX, Agostini D, Taussig M, Taylor CF, Bottomley SP, Villaverde A, de Marco A.

Stand Genomic Sci. 2011 Nov 30;5(2):195-7. doi: 10.4056/sigs.1834511. Epub 2011 Nov 22. No abstract available.


Methods to measure the kinetics of protease inhibition by serpins.

Horvath AJ, Lu BG, Pike RN, Bottomley SP.

Methods Enzymol. 2011;501:223-35. doi: 10.1016/B978-0-12-385950-1.00011-0.


Intracellular production of recombinant serpins in yeast.

Kaiserman D, Hitchen C, Levina V, Bottomley SP, Bird PI.

Methods Enzymol. 2011;501:1-12. doi: 10.1016/B978-0-12-385950-1.00001-8.


Discovery of amino acid motifs for thrombin cleavage and validation using a model substrate.

Ng NM, Pierce JD, Webb GI, Ratnikov BI, Wijeyewickrema LC, Duncan RC, Robertson AL, Bottomley SP, Boyd SE, Pike RN.

Biochemistry. 2011 Dec 6;50(48):10499-507. doi: 10.1021/bi201333g. Epub 2011 Nov 11.


A participatory learning approach to biochemistry using student authored and evaluated multiple-choice questions.

Bottomley S, Denny P.

Biochem Mol Biol Educ. 2011 Sep-Oct;39(5):352-61. doi: 10.1002/bmb.20526.


The rate of polyQ-mediated aggregation is dramatically affected by the number and location of surrounding domains.

Robertson AL, Bate MA, Buckle AM, Bottomley SP.

J Mol Biol. 2011 Nov 4;413(4):879-87. doi: 10.1016/j.jmb.2011.09.014. Epub 2011 Sep 16.


Structural change in β-sheet A of Z α(1)-antitrypsin is responsible for accelerated polymerization and disease.

Knaupp AS, Bottomley SP.

J Mol Biol. 2011 Nov 4;413(4):888-98. doi: 10.1016/j.jmb.2011.09.013. Epub 2011 Sep 16.


The structural diversity in α1-antitrypsin misfolding.

Bottomley SP.

EMBO Rep. 2011 Sep 30;12(10):983-4. doi: 10.1038/embor.2011.187. No abstract available.


Sideroblastic anemia, iron overload, and ALAS2 R452S in African-American males: phenotype and genotype features of five unrelated patients.

Lee PL, Reid TJ 3rd, Bottomley SS, Barton JC.

Am J Hematol. 2011 Sep;86(9):787-9. doi: 10.1002/ajh.22088. Epub 2011 Jul 28. No abstract available.


Flanking domain stability modulates the aggregation kinetics of a polyglutamine disease protein.

Saunders HM, Gilis D, Rooman M, Dehouck Y, Robertson AL, Bottomley SP.

Protein Sci. 2011 Oct;20(10):1675-81. doi: 10.1002/pro.698. Epub 2011 Aug 18.


Refolding your protein with a little help from REFOLD.

Phan J, Yamout N, Schmidberger J, Bottomley SP, Buckle AM.

Methods Mol Biol. 2011;752:45-57. doi: 10.1007/978-1-60327-223-0_4.


Amyloid formation from an α-helix peptide bundle is seeded by 3(10)-helix aggregates.

Singh Y, Sharpe PC, Hoang HN, Lucke AJ, McDowall AW, Bottomley SP, Fairlie DP.

Chemistry. 2011 Jan 3;17(1):151-60. doi: 10.1002/chem.201002500. Epub 2010 Dec 15.


The subtilisin-like protease AprV2 is required for virulence and uses a novel disulphide-tethered exosite to bind substrates.

Kennan RM, Wong W, Dhungyel OP, Han X, Wong D, Parker D, Rosado CJ, Law RH, McGowan S, Reeve SB, Levina V, Powers GA, Pike RN, Bottomley SP, Smith AI, Marsh I, Whittington RJ, Whisstock JC, Porter CJ, Rood JI.

PLoS Pathog. 2010 Nov 24;6(11):e1001210. doi: 10.1371/journal.ppat.1001210.


PolyQ: a database describing the sequence and domain context of polyglutamine repeats in proteins.

Robertson AL, Bate MA, Androulakis SG, Bottomley SP, Buckle AM.

Nucleic Acids Res. 2011 Jan;39(Database issue):D272-6. doi: 10.1093/nar/gkq1100. Epub 2010 Nov 8.


The folding pathway of alpha1-antitrypsin: avoiding the unavoidable.

Bottomley SP.

Proc Am Thorac Soc. 2010 Nov;7(6):404-7. doi: 10.1513/pats.201004-031AW. Review.


Identification and characterization of a misfolded monomeric serpin formed at physiological temperature.

Pearce MC, Powers GA, Feil SC, Hansen G, Parker MW, Bottomley SP.

J Mol Biol. 2010 Oct 29;403(3):459-67. doi: 10.1016/j.jmb.2010.09.007. Epub 2010 Sep 17.


Towards the treatment of polyglutamine diseases: the modulatory role of protein context.

Robertson AL, Bottomley SP.

Curr Med Chem. 2010;17(27):3058-68. Review.


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