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2023 Papers

BAP1 loss is associated with higher ASS1 expression in epithelioid mesothelioma: implications for therapeutic stratification. Barnett SE, Kenyani J, Tripari M, Butt Z, Grosman R, Querques F, Shaw L, Silva LC, Goate Z, Marciniak SJ, Rassl DM, Jackson R, Lian L-Y, Szlosarek PW, Sacco JJ, Coulson JM (2023). Mol Cancer Res doi: 10.1158/1541-7786.MCR-22-0635 [online ahead of print]. PMID: 36669126

Effects of sleep disturbance on dyspnoea and impaired lung function following hospital admission due to COVID-19 in the UK: a prospective multicentre cohort study. Jackson C, Stewart ID, Plekhanova T, Cunningham PS, Hazel AL, Al-Sheklly B, Aul R, Bolton CE, Chalder T, Chalmers JD, Chaudhuri N, Docherty AB, Donaldson G, Edwardson CL, Elneima O, Greening NJ, Hanley NA, Harris VC, Harrison EM, Ho LP, Houchen-Wolloff L, Howard LS, Jolley CJ, Jones MG, Leavy OC, Lewis KE, Lone NI, Marks M, McAuley HJC, McNarry MA, Patel BV, Piper-Hanley K, Poinasamy K, Raman B, Richardson M, Rivera-Ortega P, Rowland-Jones SL, Rowlands AV, Saunders RM, Scott JT, Sereno M, Shah AM, Shikotra A, Singapuri A, Stanel SC, Thorpe M, Wootton DG, Yates T, Gisli Jenkins R, Singh SJ, Man WD, Brightling CE, Wain LV, Porter JC, Thompson AAR, Horsley A, Molyneaux PL, Evans RA, Jones SE, Rutter MK, Blaikley JF; PHOSP-COVID Study Collaborative Group^#. (2023). Lancet Respir Med. 2023 Apr 14:S2213-2600(23)00124-8. doi: 10.1016/S2213-2600(23)00124-8. [online ahead of print]. ^#Includes Marciniak S PMID: 37072018

Prevalence of physical frailty, including risk factors, up to 1 year after hospitalisation for COVID-19 in the UK: a multicentre, longitudinal cohort study. McAuley HJC, Evans RA, Bolton CE, Brightling CE, Chalmers JD, Docherty AB, Elneima O, Greenhaff PL, Gupta A, Harris VC, Harrison EM, Ho LP, Horsley A, Houchen-Wolloff L, Jolley CJ, Leavy OC, Lone NI, Man WD, Marks M, Parekh D, Poinasamy K, Quint JK, Raman B, Richardson M, Saunders RM, Sereno M, Shikotra A, Singapuri A, Singh SJ, Steiner M, Tan AL, Wain LV, Welch C, Whitney J, Witham MD, Lord J, Greening NJ; PHOSP-COVID Study Collaborative Group^#. (2023). EClinicalMedicine. 2023 Mar 11;57:101896. doi: 10.1016/j.eclinm.2023.101896. eCollection 2023 Mar. ^#Includes Marciniak S. PMID: 36936404

SARS-CoV-2-specific nasal IgA wanes 9 months after hospitalisation with COVID-19 and is not induced by subsequent vaccination. Liew F, Talwar S, Cross A, Willett BJ, Scott S, Logan N, Siggins MK, Swieboda D, Sidhu JK, Efstathiou C, Moore SC, Davis C, Mohamed N, Nunag J, King C, Thompson AAR, Rowland-Jones SL, Docherty AB, Chalmers JD, Ho LP, Horsley A, Raman B, Poinasamy K, Marks M, Kon OM, Howard L, Wootton DG, Dunachie S, Quint JK, Evans RA, Wain LV, Fontanella S, de Silva TI, Ho A, Harrison E, Baillie JK, Semple MG, Brightling C, Thwaites RS, Turtle L, Openshaw PJM; ISARIC4C Investigators; PHOSP-COVID collaborative group^# (2023). EBioMedicine. 2023 Jan;87:104402. doi: 10.1016/j.ebiom.2022.104402. Epub 2022 Dec 19. ^#Includes Marciniak S. PMID: 36543718

2022 Papers

Z-⍺1-antitrypsin polymers impose molecular filtration in the endoplasmic reticulum after undergoing phase transition to a solid state. Chambers JE, Zubkov N, Kubánková M, Nixon-Abell J, Mela I, Abreu S, Schwiening M, Lavarda G, López-Duarte I, Dickens JA, Torres T., Kaminski CF, Holt LJ, Avezov E., Huntington JA, St George Hyslop P, Kuimova MK, Marciniak SJ. (2022). Science Advances 8:eabm2094. doi: 10.1126/sciadv.abm2094. PMID: 35394846.

Novel insights into surfactant protein C trafficking revealed through the study of a pathogenic mutant. Dickens JA, Rutherford E, Abreu S, Chambers JE, Ellis M, van Schadewijk A, Hiemstra P, Marciniak SJ (2022). Eur Respir J. 59: 2100267; doi: 10.1183/13993003.00267-2021. PMID: 34049951

Pharmacological targeting of endoplasmic reticulum stress in disease. Marciniak SJ, Chambers JE, Ron D (2022). Pharmacological targeting of endoplasmic reticulum stress in disease. Nat Rev Drug Discov. 21: 115-140. doi: 10.1038/s41573-021-00320-3. PMID: 34702991

Different cytokine patterns in BMPR2-mutation positive patients and pulmonary arterial hypertension patients without mutations and their influence on survival. Schwiening M, Swietlik E, Pandya D, Burling K, Barker P, Feng O, Treacy C, Abreu S, Wort S, Pepke-Zaba J, Gräf S, Marciniak SJ, Morrell N, Soon E (2022). Chest Jan 18:S0012-3692(22)00058-7. doi: 10.1016/j.chest.2022.01.019. [online ahead of print]. PMID: 35063447

Seventh BHD international symposium: recent scientific and clinical advancement. Woodford MR, Andreou A, Baba M, van de Beek I, Malta CD, Glykofridis I, Grimes H, Henske EP, Iliopoulos O, Kurihara M, Lazor R, Linehan WM, Matsumoto K, Marciniak SJ, Namba Y, Pause A, Rajan N, Ray A, Schmidt LS, Shi W, Steinlein OK, Thierauf J, Zoncu R, Webb A, Mollapour M. (2022) Oncotarget. 2022 13:173-181. doi: 10.18632/oncotarget.28176. eCollection 2022. PMID: 35070081

Combining clinical, radiological and genetic approaches to pneumothorax management. Grimes HL, Holden S, Babar J, Karia S, Wetscherek MTA, Barker A, Herre J, Knolle M, Maher ER, Genomics England Research Consortium and Marciniak SJ (2022). Combining clinical, radiological and genetic approaches to pneumothorax management. Thorax. 77(2):196-198. doi: thoraxjnl-2021-217210. PMID: 34145047

Meta-analysis of the association between emphysematous change on thoracic computerised tomography scan and recurrent pneumothorax. Girish M, Pharoah PD, Marciniak SJ (2022). Meta-analysis of the association between emphysematous change on thoracic computerised tomography scan and recurrent pneumothorax. QJM 115:215-221. doi: 10.1093/qjmed/hcab020. PMID: 33538832

Catamenial pneumothorax. Kardaman N, Nizami M, Marciniak S, Hogan J, Aresu G (2021). Ann R Coll Surg Engl. 104(4):e109-e112. doi: 10.1308/rcsann.2021.0164. PMID: 34825583

The role of impulse oscillometry in the management of asthma when forced expiratory maneuvers are contraindicated: case series and literature review. Jordon LH, Gore RB, Rusk RA, Knox-Brown B, Marciniak SJ. J Asthma 59:1577-1583. doi: 10.1080/02770903.2021.1941088. PMID: 34112043

Clinical characteristics with inflammation profiling of long COVID and association with 1-year recovery following hospitalisation in the UK: a prospective observational study. PHOSP-COVID Collaborative Group^#. (2022) Lancet Respir Med. 10:761-775. doi: 10.1016/S2213-2600(22)00127-8. ^#Includes Marciniak S. PMID: 35472304.

Large scale clinical trials – lessons from the COVID-19 pandemic. Horsley A, Pearmain L, Knight S, Schindler N, Wang R, Bennett M, Robey RC, Davies JC, Djukanovic R, Heaney LG, Hussell T, Marciniak SJ, McGarvey L, Porter JC, Wilkinson T, Brightling C, Ho L-P (2022). BMJ Open Respiratory Research 2022;9:doi: 10.1136/bmjresp-2022-001226 PMID: 35701071

Full-length antithrombim frameshift variant with aberrant C-terminus causes endoplasmic reticulum retention with a dominant-negative effect. Bravo-Pérez C, Toderici M, Chambers JE, Martínez-Menárguez JA, Garrido-Rodriguez P, Pérez-Sanchez H, Morena-Barrio B, Padilla J, Miñano A, Cifuentes-Riquelme R, Vicente V, Lozano ML, Marciniak SJ, de la Morena-Barrio ME, Corral J (2022). JCI Insight 7(19):e161430. doi: 10.1172/jci.insight.161430. PMID: 36214221

2021 Papers

Ambulatory thoracoscopic pleurodesis combined with indwelling pleural catheter in malignant pleural effusion. Foo CT, Pulimood T, Knolle M, Marciniak SJ, Herre J (2021). Front Surg. doi: 10.3389/fsurg.2021.738719. PMID: 34760917

A systematic review assessing the existence of pneumothorax-only variants of FLCN. Implications for lifelong surveillance of renal tumours. Matsumoto K, Lim D, Pharoah P, Maher E, Marciniak SJ (2021). Eur J Hum Genet 29(11):1595-1600. doi: 10.1038/s41431-021-00921-x. PMID: 34267338

Physical, cognitive, and mental health impacts of COVID-19 after hospitalisation (PHOSP-COVID): a UK multicentre, prospective cohort study.  Evans RA, McAuley H, Harrison EM, Shikotra A, Singapuri A, Sereno M, Elneima O, Docherty AB, Lone NI, Leavy OC, Daines L, Baillie JK, Brown JS, Chalder T, De Soyza A, Diar Bakerly N, Easom N, Geddes JR, Greening NJ, Hart N, Heaney LG, Heller S, Howard L, Hurst JR, Jacob J, Jenkins RG, Jolley C, Kerr S, Kon OM, Lewis K, Lord JM, McCann GP, Neubauer S, Openshaw PJM, Parekh D, Pfeffer P, Rahman NM, Raman B, Richardson M, Rowland M, Semple MG, Shah AM, Singh SJ, Sheikh A, Thomas D, Toshner M, Chalmers JD, Ho LP, Horsley A, Marks M, Poinasamy K, Wain LV, Brightling CE; PHOSP-COVID Collaborative Group#. (2021). Lancet Respir Med. 9(11):1275-1287. doi: 10.1016/S2213-2600(21)00383-0. #Includes Marciniak S. PMID: 34627560

The Role of Unfolded Proteins in Lung Disease. Bradley KL, Stokes CA, Marciniak SJ*, Parker LC*, Condliffe AM* (2021). Thorax 76(1):92-99. doi: 10.1136/thoraxjnl-2019-213738. *Joint senior

A solution scan of societal options to reduce transmission and spread of respiratory viruses: SARS-CoV-2 as a case study. Sutherland WJ, Taylor NG, Aldridge DC, Martin P, Rhodes C, Shackelford G, Beard S, Belfield H, Bladon AJ, Brick C, Christie AP, Dobson AP, Downey H, Hood ASC, Hua F, Hughes AC, Jarvis RM, MacFarlane D, Morgan WH, Mupepele AC, Marciniak SJ, Nelson C, Ó hÉigeartaigh S, Rios Rojas C, Sainsbury KA, Smith RK, Sundaram LS, Thornton A, Watkins J, White TB, Willott K, Petrovan SO. J Biosaf Biosecur. 2021 Dec;3(2):84-90. doi: 10.1016/j.jobb.2021.08.003. Epub 2021 Sep 15. PMID: 34541465

Biological basis for novel mesothelioma therapies. Obacz J, Yung H, Shamseddin M, Linnane E, Liu X, Azad AA, Rassl DM, Fairen-Jimenez D, Rintoul RC, Nikolić MZ, Marciniak SJ. Br J Cancer. 2021 Oct;125(8):1039-1055. doi: 10.1038/s41416-021-01462-2. Epub 2021 Jul 5. PMID: 34226685

Novel insights into surfactant protein C trafficking revealed through the study of a pathogenic mutant. Dickens JA, Rutherford EN, Abreu S, Chambers JE, Ellis MO, van Schadewijk A, Hiemstra PS, Marciniak SJ. Eur Respir J. 2021 May 28:2100267. doi: 10.1183/13993003.00267-2021. Online ahead of print. PMID: 34049951

Development of a small molecule that corrects misfolding and increases secretion of Z α₁ -antitrypsin. Lomas DA, Irving JA, Arico-Muendel C, Belyanskaya S, Brewster A, Brown M, Chung CW, Dave H, Denis A, Dodic N, Dossang A, Eddershaw P, Klimaszewska D, Haq I, Holmes DS, Hutchinson JP, Jagger AM, Jakhria T, Jigorel E, Liddle J, Lind K, Marciniak SJ, Messer J, Neu M, Olszewski A, Ordonez A, Ronzoni R, Rowedder J, Rüdiger M, Skinner S, Smith KJ, Terry R, Trottet L, Uings I, Wilson S, Zhu Z, Pearce AC. EMBO Mol Med e13167 https://doi.org/10.15252/emmm.202013167. PMID: 33512066

2020 Papers

Role of unfolded proteins in lung disease. Bradley KL, Stokes CA, Marciniak SJ, Parker LC, Condliffe AM. Thorax. 2021 Jan;76(1):92-99. doi: 10.1136/thoraxjnl-2019-213738. Epub 2020 Oct 19. PMID: 33077618 Free PMC article.

The integrated stress response in pulmonary disease.Emanuelli G, Nassehzadeh-Tabriz N, Morrell NW, Marciniak SJ. Eur Respir Rev. 2020 Oct 1;29(157):200184. doi: 10.1183/16000617.0184-2020. Print 2020 Sep 30. PMID: 33004527 Free PMC article.

COVID-19 and pneumothorax: a multicentre retrospective case series. Martinelli AW, Ingle T, Newman J, Nadeem I, Jackson K, Lane ND, Melhorn J, Davies HE, Rostron AJ, Adeni A, Conroy K, Woznitza N, Matson M, Brill SE, Murray J, Shah A, Naran R, Hare SS, Collas O, Bigham S, Spiro M, Huang MM, Iqbal B, Trenfield S, Ledot S, Desai S, Standing L, Babar J, Mahroof R, Smith I, Lee K, Tchrakian N, Uys S, Ricketts W, Patel ARC, Aujayeb A, Kokosi M, Wilkinson AJK, Marciniak SJ. Eur Respir J. 2020 Nov 19;56(5):2002697. doi: 10.1183/13993003.02697-2020. Print 2020 Nov. PMID: 32907891.

Tissue expression of lactate transporters (MCT1 and MCT4) and prognosis of malignant pleural mesothelioma. Dell’Anno I, Barone E, Mutti L, Rassl DM, Marciniak SJ, Silvestri R, Landi S, Gemignani F. J Transl Med. 2020 Sep 4;18(1):341. doi: 10.1186/s12967-020-02487-6. PMID: 32887638.

HaloFlippers: A General Tool for the Fluorescence Imaging of Precisely Localized Membrane Tension Changes in Living Cells. Straková K, López-Andarias J, Jiménez-Rojo N, Chambers JE, Marciniak SJ, Riezman H, Sakai N, Matile S. ACS Cent Sci. 2020 Aug 26;6(8):1376-1385. doi: 10.1021/acscentsci.0c00666. Epub 2020 Jul 20. PMID: 32875078.

Pneumothorax and the biology of Birt-Hogg-Dubé syndrome.Marciniak SJ, Johnson SR. Thorax. 2020 Jun;75(6):442-443. doi: 10.1136/thoraxjnl-2020-214861. PMID: 32414881.

Susceptibility to cellular stress in PS1 mutant N2a cells is associated with mitochondrial defects and altered calcium homeostasis. Rojas-Charry L, Calero-Martinez S, Morganti C, Morciano G, Park K, Hagel C, Marciniak SJ, Glatzel M, Pinton P, Sepulveda-Falla D. Sci Rep. 2020 Apr 15;10(1):6455. doi: 10.1038/s41598-020-63254-7. PMID: 32296078.

2019 Papers

Linker length affects photostability of protein-targeted sensor of cellular microviscosity. Kubánková M, Chambers JE, Huber RG, Bond PJ, Marciniak SJ, Kuimova MK. Methods Appl Fluoresc. 2019 Oct 10;7(4):044004. doi: 10.1088/2050-6120/ab481f. PMID: 31557750

Proteostasis During Cerebral Ischemia. Thiebaut AM, Hedou E, Marciniak SJ, Vivien D, Roussel BD. Front Neurosci. 2019 Jun 19;13:637. doi: 10.3389/fnins.2019.00637. eCollection 2019. PMID: 31275110 Free PMC article.

Inactivation of Ppp1r15a minimises weight gain and insulin resistance during caloric excess in female mice. Patel V, Bidault G, Chambers JE, Carobbio S, Everden AJT, Garcés C, Dalton LE, Gribble FM, Vidal-Puig A, Marciniak SJ. Sci Rep. 2019 Feb 27;9(1):2903. doi: 10.1038/s41598-019-39562-y. PMID: 30814564.

The Genetics of Pneumothorax. Boone PM, Scott RM, Marciniak SJ, Henske EP, Raby BA. Am J Respir Crit Care Med. 2019 Jun 1;199(11):1344-1357. doi: 10.1164/rccm.201807-1212CI. PMID: 30681372.

Endoplasmic reticulum stress: a key player in human disease. Marciniak SJ. FEBS J. 2019 Jan;286(2):228-231. doi: 10.1111/febs.14740. PMID: 30677245.

2018 Papers

An Optical Technique for Mapping Microviscosity Dynamics in Cellular Organelles. Chambers J.E., Kubankova M., Huber, R., López-Duart I, Avezov E., Bond P., Marciniak S.J.*, Kuimova, M.* (2018).  ACS Nano. doi: 10.1021/acsnano.8b00177 [Epub ahead of print] *Joint senior authors

Single particle trajectories reveal active endoplasmic reticulum luminal flow. Holcman D, Parutto P, Chambers JE, Fantham M, Young LJ, Marciniak SJ, Kaminski CF, Ron D, Avezov E. Nat Cell Biol. 2018 Oct;20(10):1118-1125. doi: 10.1038/s41556-018-0192-2. Epub 2018 Sep 17.

hiPSC hepatocyte model demonstrates the role of unfolded protein response and inflammatory networks in α₁-antitrypsin deficiency. Segeritz CP, Rashid ST, de Brito MC, Serra MP, Ordonez A, Morell CM, Kaserman JE, Madrigal P, Hannan NRF, Gatto L, Tan L, Wilson AA, Lilley K, Marciniak SJ, Gooptu B, Lomas DA, Vallier L. J Hepatol. 2018 Oct;69(4):851-860. doi: 10.1016/j.jhep.2018.05.028.

The integrated stress response regulates BMP signaling through effects on translation. Malzer E., Dominicus C.S., Chambers J.E., Dickens J.A., Mookerjee S., Marciniak S.J.(2018).  BMC Biology 16:34 https://doi.org/10.1186/s12915-018-0503-x

Measuring the effects of α₁ -antitrypsin polymerisation on the structure and biophysical properties of the endoplasmic reticulum. Chambers JE, Dickens JA, Marciniak SJ. Biol Cell. 2018 Nov;110(11):249-255. doi: 10.1111/boc.201800023. Epub 2018 Sep 10.

Familial pneumothorax – towards precision medicine. Scott R.M., Henske E.P., Raby B., Boone P.M., Rusk R.A., Marciniak S.J. (2018). Thorax 73:270-276.

Pulmonary endoplasmic reticulum stress: Scars, Smoke and Suffocation. Dickens J.A., Malzer E., Chambers J.E. Marciniak S.J. (2018).  FEBS J doi: 10.1111/febs.14381.

2017 Papers

PPP1R15A-mediated dephosphorylation of eIF2α is unaffected by Sephin1 or Guanabenz. Crespillo-Casado A., Chambers J.E., Fischer P., Marciniak S.J., Ron D. (2017). eLIFE. 10.7554/eLife.19893.

Endoplasmic reticulum stress in lung disease. Marciniak S.J. (2017). Eur. Respir. Rev. 26: 170018 [https://doi.org/10.1183/16000617.0018-2017].

2016 Papers

The endoplasmic reticulum remains functionally connected by vesicular transport after its fragmentation in cells expressing Z-α1-antitrypsin. Dickens J.A., Ordóñez A., Chambers J.E., Beckett A.J., Patel V., Malzer E., Dominicus C.S., Bradley J., Peden A.A., Prior I.A., Lomas D.A., Marciniak S.J. (2016). FASEB J. 30:4083-4097.

New Concepts in Alpha-1 Antitrypsin Deficiency Disease Mechanisms. Marciniak S.J., Ordóñez A., Dickens J.A., Chambers J.E., Patel V., Dominicus C.S., Malzer E. (2016). Ann Am Thorac Soc. 13 Suppl 4:S289-96. doi: 10.1513/AnnalsATS.201506-358KV.

α1-Antitrypsin deficiency. Greene C.M., Marciniak S.J., Teckman J., Ferrarotti I., Brantly M.L., Lomas D.A., Stoller J.K., McElvaney N.G. (2016). Nat Rev Dis Primers. 2:16051. doi: 10.1038/nrdp.2016.51.

Polymers of Z α1-antitrypsin are secreted in cell models of disease. Fra A., Cosmi F., Ordoñez A., Berardelli R., Perez J., Guadagno N.A., Corda L., Marciniak S.J., Lomas D.A., Miranda E. (2016). Eur Respir J. 47:1005-9. doi: 10.1183/13993003.00940-2015.

2015 Papers

Virulence factors of Pseudomonas aeruginosa induce both the unfolded protein and integrated stress responses in airway epithelial cells.van ‘t Wout E.F.A., van Schadewijk A., van Boxtel R., Dalton L.E., Clarke H.J., Tommassen J., Marciniak S.J.*, Hiemstra P.S.* (2015). ­­PLoS Pathogens 11(6): e1004946. doi:10.1371/journal.ppat.1004946 *Joint senior authors

Actin dynamics tune the integrated stress response by regulatins eukaryotic initiation factor 2α dephosphorylation. Chambers, J.E., Dalton, L.E., Clarke, H.J., Malzer, E., Dominicus, C.S., Patel, V., Moorhead, G.B.G., Ron, D., and Marciniak, S.J. (2015). eLIFE doi.org/10.7554/eLife.04872.

G-actin provides substrate-specificity to eukaryotic initiation factor 2a holophosphatase. Chen, R., Rato, C., Yan, Y., Crespillo-Casado, A., Clarke, H.J., Harding, H.P., Marciniak, S.J. *, Read, R.J. *, and Ron, D*. (2015). eLIFE doi.org/10.7554/eLife.04871. *Joint senior authors

Spontaneous pneumothorax can be associated with TGFBR2 mutation. Chambers J.E., Dalton L.E., Subramanian D.N., Gooptu B., Balan A., Park S.M., Holden S., Marciniak S.J. (2015) Eur Respir J. 46:1832-5. doi: 10.1183/13993003.00952-2015.

Non-interventional statistical comparison of BTS and CHEST guidelines for size and severity in primary pneumothorax. Nikolic M.Z., Lok L.S.C., Mattishent K., Barth S., Yung B., Cummings N., Wade D., Vali Y., Chong K., Wilkinson A., Mikolasch T., Brij S., Jenkins H.S., Kamath A., Pasteur M., Hopkins T., Wason J., Marciniak S.J. (2015) Eur. Respir. J. 45: 1731-4.

A single-chain variable fragment intrabody prevents intracellular polymerisation of Z alpha₁-antitrypsin while allowing its antiproteinase activity. Ordóñez A., Pérez J., Tan L., Dickens J.A., Motamedi-Shad N., Irving J.A., Haq I., Ekeowa U., Marciniak S.J., Miranda E., Lomas D.A. (2015) FASEB J. 29:2667-78.

Characterising the association of latency with α(1)-antitrypsin polymerisation using a novel monoclonal antibody. Tan L., Perez J., Mela M., Miranda E., Burling K.A., Rouhani F.N., DeMeo D.L., Haq I., Irving J.A., Ordonez A., Dickens J.A., Brantly M., Marciniak S.J., Alexander G., Gooptu B., Lomas D.A., (2015) Int J Biochem Cell Biol. 58:81-91.

Function of monocytes and monocyte-derived macrophages in α₁-antitrypsin deficiency. van ‘t Wout E.F.A., van Schadewijk A., Lomas D.A., Stolk J, Marciniak S.J., Hiemstra P.S. (2015). Eur. Respir. J. 45: 3654-376.

2014 Papers

The TRiC/CCT Chaperone Is Implicated in Alzheimer’s Disease Based on Patient GWAS and an RNAi Screen in Aβ-Expressing Caenorhabditis elegans. Khabirova E., Moloney A., Marciniak S.J., Williams J., Lomas D.A., Oliver S.G., Favrin G., Sattelle D.B., Crowther D.C. (2014).    PLoS One. 2014 Jul 31;9(7):e102985. doi: 10.1371/journal.pone.0102985.eCollection 2014.

Palliative treatment for symptomatic malignant pericardial effusion. Jama G.M., Scarci M., Bowden J., & Marciniak S.J. (2014)  Interact Cardiovasc Thorac Surg. 2014 Dec;19(6):1019-26. doi: 10.1093/icvts/ivu267.

Protein misfolding and ER stress. Chambers JE, Marciniak SJ. (2014) Am J Physiol Cell Physiol. 15;307(8):C657-70. doi: 10.1152/ajpcell.00183.2014.

Endoplasmic reticulum stress in malignancy. Clarke HJ, Chambers JE, Liniker E, Marciniak SJ. Cancer Cell. 2014 May 12;25(5):563-73. doi: 10.1016/j.ccr.2014.03.015.

The unfolded protein response governs integrity of the haematopoietic stem-cell pool during stress.  van Galen P, Kreso A, Mbong N, Kent DG, Fitzmaurice T, Chambers JE, Xie S, Laurenti E, Hermans K, Eppert K, Marciniak SJ, Goodall JC, Green AR, Wouters BG, Wienholds E, Dick JE. Nature. 2014 Jun 12;510(7504):268-72. doi: 10.1038/nature13228.

The integrated stress response in lung disease. van ‘t Wout EF, Hiemstra PS, Marciniak SJ. Am J Respir Cell Mol Biol. 2014 Jun;50(6):1005-9. doi: 10.1165/rcmb.2014-0019TR.

Circulating polymers in α1-antitrypsin deficiency. Tan L, Dickens JA, Demeo DL, Miranda E, Perez J, Rashid ST, Day J, Ordoñez A, Marciniak SJ, Haq I, Barker AF, Campbell EJ, Eden E, McElvaney NG, Rennard SI, Sandhaus RA, Stocks JM, Stoller JK, Strange C, Turino G, Rouhani FN, Brantly M, Lomas DA. Eur Respir J. 2014 May;43(5):1501-4. doi: 10.1183/09031936.00111213.

Genetic susceptibility. Marciniak SJ, Lomas DA. Clin Chest Med. 2014 Mar;35(1):29-38. doi: 10.1016/j.ccm.2013.10.008.

Increased ERK signalling promotes inflammatory signalling in primary airway epithelial cells expressing Z α1-antitrypsin. van ‘t Wout EF, Dickens JA, van Schadewijk A, Haq I, Kwok HF, Ordóñez A, Murphy G, Stolk J, Lomas DA, Hiemstra PS, Marciniak SJ. (2014) Hum Mol Genet. 15;23(4):929-41. doi: 10.1093/hmg/ddt487.

2013 Papers

Paneth cells as a site of origin for intestinal inflammation. Adolph TE, Tomczak MF, Niederreiter L, Ko HJ, Böck J, Martinez-Naves E, Glickman JN, Tschurtschenthaler M, Hartwig J, Hosomi S, Flak MB, Cusick JL, Kohno K, Iwawaki T, Billmann-Born S, Raine T, Bharti R, Lucius R, Kweon MN, Marciniak SJ, Choi A, Hagen SJ, Schreiber S, Rosenstiel P, Kaser A, Blumberg RS. (2013) Nature. 503(7475):272-6. doi: 10.1038/nature12599.

Suppression of Aβ toxicity by puromycin-sensitive aminopeptidase is independent of its proteolytic activity. Kruppa AJ, Ott S, Chandraratna DS, Irving JA, Page RM, Speretta E, Seto T, Camargo LM, Marciniak SJ, Lomas DA, Crowther DC. Biochim Biophys Acta. 2013 Aug 2;1832(12):2115-2126. doi: 10.1016/j.bbadis.2013.07.019.

Sterol metabolism regulates neuroserpin polymer degradation in the absence of the unfolded protein response in the dementia FENIB. Roussel BD, Newton TM, Malzer E, Simecek N, Haq I, Thomas SE, Burr ML, Lehner PJ, Crowther DC, Marciniak SJ, Lomas DA. (2013) Hum Mol Genet. 15;22(22):4616-26. doi: 10.1093/hmg/ddt310.

Chloroquine prevents progression of experimental pulmonary hypertension via inhibition of autophagy and lysosomal bone morphogenetic protein type II receptor degradation. Long L, Yang X, Southwood M, Lu J, Marciniak SJ, Dunmore BJ, Morrell NW. Circ Res. 2013 Apr 12;112(8):1159-70. doi: 10.1161/CIRCRESAHA.111.300483.

Coordinate regulation of eIF2α phosphorylation by PPP1R15 and GCN2 is required during Drosophila development.  Malzer E, Szajewska-Skuta M, Dalton LE, Thomas SE, Hu N, Skaer H, Lomas DA, Crowther DC, Marciniak SJ. (2013) J Cell Sci. 126(Pt 6):1406-15. doi: 10.1242/jcs.117614.

The endoplasmic reticulum stress marker CHOP predicts survival in malignant mesothelioma.  Dalton LE, Clarke HJ, Knight J, Lawson MH, Wason J, Lomas DA, Howat WJ, Rintoul RC, Rassl DM, Marciniak SJ. Br J Cancer. 2013 Apr 2;108(6):1340-7. doi: 10.1038/bjc.2013.66.

p53 and translation attenuation regulate distinct cell cycle checkpoints during endoplasmic reticulum (ER) stress. Thomas SE, Malzer E, Ordóñez A, Dalton LE, van ‘t Wout EF, Liniker E, Crowther DC, Lomas DA, Marciniak SJ. J Biol Chem. 2013 Mar 15;288(11):7606-17. doi: 10.1074/jbc.M112.424655.

Endoplasmic reticulum dysfunction in neurological disease. Roussel BD, Kruppa AJ, Miranda E, Crowther DC, Lomas DA, Marciniak SJ. Lancet Neurol. 2013 Jan;12(1):105-18. doi: 10.1016/S1474-4422(12)70238-7.

Evaluation of secondary spontaneous pneumothorax with multidetector CT. Hilliard NJ, Marciniak SJ, Babar JL, Balan A. Clin Radiol. 2013 May;68(5):521-8. doi: 10.1016/j.crad.2012.10.008. Epub 2012 Dec 5.

Endoplasmic reticulum polymers impair luminal protein mobility and sensitize to cellular stress in alpha1-antitrypsin deficiency.  Ordóñez A, Snapp EL, Tan L, Miranda E, Marciniak SJ, Lomas DA. Hepatology. 2013 May;57(5):2049-60. doi: 10.1002/hep.26173.

2012

Ordóñez A., Snapp E.L., Tan L., Miranda E., Marciniak S.J.§*, Lomas D.A.* (2012) Endoplasmic reticulum polymers impair luminal protein mobility and sensitise to cellular stress in α1-antitrypsin deficiency. Hepatology 2012 Nov 29. doi: 10.1002/hep.26173. [Epub ahead of print]. *Joint senior authors §Corresponding author

Dalton L.E., Healey E., Irving J. & Marciniak S.J. (2012).  Phosphoproteins in stress-induced disease.  Prog Mol Biol Transl Sci 106: 189-221

Hilliard NJ, Marciniak SJ, Babar JL, Balan A. (2013) Evaluation of secondary spontaneous pneumothorax with multidetector CT.  Clin Radiol. 2012 Dec 5. pii: S0009-9260(12)00526-0. doi: 10.1016/j.crad.2012.10.008. [Epub ahead of print]

Klionsky D.J. et al. (2012) Guidelines for the use and interpretation of assays for monitoring autophagy.  Autophagy 8: 445-544.

2011

YusaK, S. RashidT, Strick-MarchandH, VarelaI, LiuP-Q, PaschonDE, Miranda E, Ordóñez A, HannanN, Rouhani F, Darche S, AlexanderG, MarciniakSJ, Hasegawa M, Fusaki N, HolmesMC, Di SantoJP, LomasDA, BradleyA and VallierL (2011) Targeted gene correction of a₁-antitrypsin deficiency in induced pluripotent stem cells. Nature 478: 391-394.

Hopkins T.G., Maher E., Reid E. & Marciniak SJ. (2011) Recurrent pneumothorax. Lancet 377: 1624.

Jahn TR, Malzer E, Roote J, Vishnivetskaya A, Imarisio S, Giannakou M, Panser K, Marciniak S, Crowther DC.  (2011) Modeling Serpin Conformational Diseases in Drosophila melanogaster.  Methods Enzymol. 499: 227-58.

Ekeowa U.I., Marciniak S.J., & Lomas D.A. (2011). a₁-antitrypsin deficiency and inflammation. Expert Rev. Clin. Immunol. 7: 243-52.

Roussel B.D., Irving J.A., Ekeowa U.I., Belorgey D., Haq I., Ordóñez A., Kruppa A.J., Duvoix A., Rashid S.T., Crowther D.C., Marciniak S.J., Lomas D.A. (2011) Unravelling the twists and turns of the serpinopathies. FEBS J. 278: 3859-67.

Belorgey D., Irving J.A., Ekeowa U.I., Freeke J., Roussel B.D., Miranda E., Pérez J., Marciniak S.J., Crowther D.C., Michel C.H. & Lomas D.A. (2011). Characterisation of serpin polymers in vitro and in vivo.  Methods 53: 255-266.

Liu B., Moloney A., Meehan S., Morris K., Thomas S.E., Serpell L.C., Hider R., Marciniak S.J., Lomas D.A., & Crowther D.C. (2011). Iron Promotes The Toxicity Of Amyloid Beta Peptide By Impeding Its Ordered Aggregation. J Biol. Chem. 286: 4248-56.

2010

Malzer E., Daly M.L.,  Moloney A.,  Sendall T.J., Thomas, S.L. Ryder E., Ryoo H.D., Crowther D.C.,  Lomas, D.A. & Marciniak S.J. (2010). Impaired tissue growth is mediated by checkpoint kinase 1 (CHK1) in the integrated stress response.  J. Cell Sci. 123: 2892-2900.

Rashid S.T., Corbineau S., Hannan N., Marciniak S.J., Miranda E., Alexander G., Huang-Doran I., Ahrlund-Richter L., Skepper J., Griffin J., Semple R., Weber A. Lomas D.A. & Vallier L. (2010). Modeling inherited metabolic disorders of the liver with human induced pluripotent stem cells.  J Clin. Invest. 120: 3127-36.

 Miranda E., Pérez J., Ekeowa U.I., Hadzic N., Kalsheker N., Gooptu B., Portmann B., Belorgey D., Hill M., Chambers S., Teckman J., Alexander G.J., Marciniak S.J. & Lomas D.A. (2010).A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with α1-antitrypsin deficiency.  Hepatology 52: 1078-88.

Marciniak S.J. & Lomas (2010). a₁-Antitrypsin deficiency and autophagy. N Engl J Med. 363:1863-1864.

Marciniak S.J. & Ron D. (2010).  The Unfolded Protein Response in Lung Disease.  Proc. Am. Thor. Soc. 7:356-362.

Thomas S.E , Dalton L.E., Daly M.L., Malzer E., & Marciniak S.J. (2010) Diabetes as a disease of endoplasmic reticulum stress. Diabetes Metab Res Rev. 26: 611-21.

2009

Kröger H., Miranda E.,  MacLeod I., Pérez J., Crowther D.C.,  Marciniak S.J.* & Lomas D.A.* (2009).  ERAD and autophagy cooperate to degrade polymerogenic mutant serpins. J Biol. Chem. 284: 22793-802. *Joint senior authors

Davies M.J., Miranda E., Kaufman R.J., Marciniak S.J.* & Lomas D.A.* (2009). Neuroserpin polymers activate NF-kB by a calcium-signalling pathway that is independent of the unfolded protein response.  J Biol. Chem. 284: 18202-9.  *Joint senior authors

Ekeowa U.I., Gooptu B., Belorgey D., Hägglöf P., Karlsson-Li S,. Miranda E., Pérez J., MacLeod I., Kroger H., Marciniak S.J., Crowther D.C. & Lomas D.A. (2009). a₁-Antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies.  Clin Sci. 116: 837-50.

Marciniak S.J. & Lomas D.A. (2009). What can naturally occurring mutations tell us about the pathogenesis of COPD?  Thorax 64: 359-364.

2008

Marciniak S.J. & Lomas D.A. (2008).  Serpins, intracellular firewalls and necrosis.  Trends Cell Biol.  18:45-47.

2006

Marciniak S.J. & Ron D. (2006). Endoplasmic reticulum stress signalling in disease. Physiol Rev. 86: 1133-49.

MarciniakS.J., Garcia-BonillaL., HuJ., HardingH.P., RonD. (2006). Activation-dependent substrate recruitment by the eukaryotic translation initiation factor 2 kinase PERK. J Cell Biol. 172: 201-9.

Pereira R.C., Stadmeyer L., Marciniak S.J., Ron D., Canalis E. (2006). C/EBP homologous protein is necessary for normal ost­eoblastic function. J Cell Biochem. 97:633-640.

2005

Harding HP, Zhang Y, Khersonsky S, Marciniak S, Scheuner D, Kaufman RJ, Javitt N, Chang YT, Ron D. (2005). Bioactive small molecules reveal antagonism between the integrated stress response and sterol-regulated gene expression. Cell Metab. 2:361-71.

Silva R.M., Ries V., Oo T.F., Yarygina O., Jackson-Lewis V., Ryu E.J., Lu P.D., Marciniak S.J., Ron D., Przedborski S., Kholodilov N., Greene L.A., Burke R.E. (2005).  CHOP/GADD153 is a mediator of apoptotic death in substantia nigra dopamine neurons in an in vivo neurotoxin model of parkinsonism. J Neurochem. 95:974-8.

2004

Marciniak S.J, Yun CY, Oyadomari S, Novoa I, Zhang Y, Jungreis R, Nagata K, Harding HP, Ron D. (2004). CHOP induces death by promoting protein synthesis and oxidation in the stressed endoplasmic reticulum. Genes Dev. 2004 18:3066-77.

Lu P.D., Jousse C., Marciniak S.J., Zhang Y., Novoa I., Scheuner D., Kaufman R.J., Ron D., Harding H.P. (2004). Cytoprotection by pre-emptive conditional phosphorylation of translation initiation factor 2. EMBO J. 23:169-79.