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The Genetics of Pneumothorax.

Boone PM, Scott RM, Marciniak SJ, Henske EP, Raby BA.

Am J Respir Crit Care Med. 2019 Jan 25. doi: 10.1164/rccm.201807-1212CI. [Epub ahead of print]


Endoplasmic reticulum stress: a key player in human disease.

Marciniak SJ.

FEBS J. 2019 Jan;286(2):228-231. doi: 10.1111/febs.14740.


Author Correction: α1-Antitrypsin deficiency.

Greene CM, Marciniak SJ, Teckman J, Ferrarotti I, Brantly ML, Lomas DA, Stoller JK, McElvaney NG.

Nat Rev Dis Primers. 2018 Oct 29;4(1):40. doi: 10.1038/s41572-018-0043-2.


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.


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. Review.


Time Is of the Essence: A Young Man with Recurrent Pneumothorax and Cavitating Lung Lesions.

Lim R, Marciniak SJ, Marcadier J, Rassl D, Mitchell PD.

Ann Am Thorac Soc. 2018 Aug;15(8):988-991. doi: 10.1513/AnnalsATS.201712-968CC. No abstract available.


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. Epub 2018 Jun 5.


An Optical Technique for Mapping Microviscosity Dynamics in Cellular Organelles.

Chambers JE, Kubánková M, Huber RG, López-Duarte I, Avezov E, Bond PJ, Marciniak SJ, Kuimova MK.

ACS Nano. 2018 May 22;12(5):4398-4407. doi: 10.1021/acsnano.8b00177. Epub 2018 Apr 18.


The integrated stress response regulates BMP signalling through effects on translation.

Malzer E, Dominicus CS, Chambers JE, Dickens JA, Mookerjee S, Marciniak SJ.

BMC Biol. 2018 Apr 3;16(1):34. doi: 10.1186/s12915-018-0503-x.


Pulmonary endoplasmic reticulum stress-scars, smoke, and suffocation.

Dickens JA, Malzer E, Chambers JE, Marciniak SJ.

FEBS J. 2019 Jan;286(2):322-341. doi: 10.1111/febs.14381. Epub 2018 Jan 22. Review.


Familial pneumothorax: towards precision medicine.

Scott RM, Henske EP, Raby B, Boone PM, Rusk RA, Marciniak SJ.

Thorax. 2018 Mar;73(3):270-276. doi: 10.1136/thoraxjnl-2017-211169. Epub 2017 Dec 28. Review.


Endoplasmic reticulum stress in lung disease.

Marciniak SJ.

Eur Respir Rev. 2017 Jun 28;26(144). pii: 170018. doi: 10.1183/16000617.0018-2017. Print 2017 Jun 30. Review.


PPP1R15A-mediated dephosphorylation of eIF2α is unaffected by Sephin1 or Guanabenz.

Crespillo-Casado A, Chambers JE, Fischer PM, Marciniak SJ, Ron D.

Elife. 2017 Apr 27;6. pii: e26109. doi: 10.7554/eLife.26109.


Absolute Bioavailability and Pharmacokinetic Comparability of Sirukumab Following Subcutaneous Administration by a Prefilled Syringe or an Autoinjector.

Zhuang Y, de Vries DE, Marciniak SJ, Liu H, Zhou H, Davis HM, Leon F, Raible D, Xu Z.

Clin Pharmacol Drug Dev. 2017 Nov;6(6):570-576. doi: 10.1002/cpdd.328. Epub 2017 Jan 3.


The endoplasmic reticulum remains functionally connected by vesicular transport after its fragmentation in cells expressing Z-α1-antitrypsin.

Dickens JA, Ordóñez A, Chambers JE, Beckett AJ, Patel V, Malzer E, Dominicus CS, Bradley J, Peden AA, Prior IA, Lomas DA, Marciniak SJ.

FASEB J. 2016 Dec;30(12):4083-4097. Epub 2016 Sep 6.


New Concepts in Alpha-1 Antitrypsin Deficiency Disease Mechanisms.

Marciniak SJ, Ordóñez A, Dickens JA, Chambers JE, Patel V, Dominicus CS, Malzer E.

Ann Am Thorac Soc. 2016 Aug;13 Suppl 4:S289-96. doi: 10.1513/AnnalsATS.201506-358KV. Review.


First-in-human study to assess guselkumab (anti-IL-23 mAb) pharmacokinetics/safety in healthy subjects and patients with moderate-to-severe psoriasis.

Zhuang Y, Calderon C, Marciniak SJ Jr, Bouman-Thio E, Szapary P, Yang TY, Schantz A, Davis HM, Zhou H, Xu Z.

Eur J Clin Pharmacol. 2016 Nov;72(11):1303-1310. Epub 2016 Aug 11.


α1-Antitrypsin deficiency.

Greene CM, Marciniak SJ, Teckman J, Ferrarotti I, Brantly ML, Lomas DA, Stoller JK, McElvaney NG.

Nat Rev Dis Primers. 2016 Jul 28;2:16051. doi: 10.1038/nrdp.2016.51. Review. Erratum in: Nat Rev Dis Primers. 2018 Oct 29;4(1):40.


Polymers of Z α1-antitrypsin are secreted in cell models of disease.

Fra A, Cosmi F, Ordoñez A, Berardelli R, Perez J, Guadagno NA, Corda L, Marciniak SJ, Lomas DA, Miranda E.

Eur Respir J. 2016 Mar;47(3):1005-9. doi: 10.1183/13993003.00940-2015. Epub 2016 Feb 4. No abstract available.


Autism-associated R451C mutation in neuroligin3 leads to activation of the unfolded protein response in a PC12 Tet-On inducible system.

Ulbrich L, Favaloro FL, Trobiani L, Marchetti V, Patel V, Pascucci T, Comoletti D, Marciniak SJ, De Jaco A.

Biochem J. 2016 Feb 15;473(4):423-34. doi: 10.1042/BJ20150274. Epub 2015 Nov 30.


Spontaneous pneumothorax can be associated with TGFBR2 mutation.

Chambers JE, Dalton LE, Subramanian DN, Gooptu B, Balan A, Park SM, Holden S, Marciniak SJ.

Eur Respir J. 2015 Dec;46(6):1832-5. doi: 10.1183/13993003.00952-2015. Epub 2015 Oct 22. No abstract available.


MesobanK UK: an international mesothelioma bioresource.

Rintoul RC, Rassl DM, Gittins J, Marciniak SJ; MesobanK collaborators.

Thorax. 2016 Apr;71(4):380-2. doi: 10.1136/thoraxjnl-2015-207496. Epub 2015 Oct 14.


Interactions between N-linked glycosylation and polymerisation of neuroserpin within the endoplasmic reticulum.

Moriconi C, Ordoñez A, Lupo G, Gooptu B, Irving JA, Noto R, Martorana V, Manno M, Timpano V, Guadagno NA, Dalton L, Marciniak SJ, Lomas DA, Miranda E.

FEBS J. 2015 Dec;282(23):4565-79. doi: 10.1111/febs.13517. Epub 2015 Oct 3.


Virulence Factors of Pseudomonas aeruginosa Induce Both the Unfolded Protein and Integrated Stress Responses in Airway Epithelial Cells.

van 't Wout EF, van Schadewijk A, van Boxtel R, Dalton LE, Clarke HJ, Tommassen J, Marciniak SJ, Hiemstra PS.

PLoS Pathog. 2015 Jun 17;11(6):e1004946. doi: 10.1371/journal.ppat.1004946. eCollection 2015 Jun.


Evaluation of disease-mediated therapeutic protein-drug interactions between an anti-interleukin-6 monoclonal antibody (sirukumab) and cytochrome P450 activities in a phase 1 study in patients with rheumatoid arthritis using a cocktail approach.

Zhuang Y, de Vries DE, Xu Z, Marciniak SJ Jr, Chen D, Leon F, Davis HM, Zhou H.

J Clin Pharmacol. 2015 Dec;55(12):1386-94. doi: 10.1002/jcph.561. Epub 2015 Jul 29.


Noninterventional statistical comparison of BTS and CHEST guidelines for size and severity in primary pneumothorax.

Nikolić MZ, Lok LS, Mattishent K, Barth S, Yung B, Cummings NM, Wade D, Vali Y, Chong K, Wilkinson A, Mikolasch T, Brij S, Jenkins HS, Kamath AV, Pasteur M, Hopkins TG, Wason J, Marciniak SJ.

Eur Respir J. 2015 Jun;45(6):1731-4. doi: 10.1183/09031936.00118614. Epub 2015 Mar 18. No abstract available.


G-actin provides substrate-specificity to eukaryotic initiation factor 2α holophosphatases.

Chen R, Rato C, Yan Y, Crespillo-Casado A, Clarke HJ, Harding HP, Marciniak SJ, Read RJ, Ron D.

Elife. 2015 Mar 16;4. doi: 10.7554/eLife.04871.


Actin dynamics tune the integrated stress response by regulating eukaryotic initiation factor 2α dephosphorylation.

Chambers JE, Dalton LE, Clarke HJ, Malzer E, Dominicus CS, Patel V, Moorhead G, Ron D, Marciniak SJ.

Elife. 2015 Mar 16;4. doi: 10.7554/eLife.04872.


A single-chain variable fragment intrabody prevents intracellular polymerization of Z α1-antitrypsin while allowing its antiproteinase activity.

Ordóñez A, Pérez J, Tan L, Dickens JA, Motamedi-Shad N, Irving JA, Haq I, Ekeowa U, Marciniak SJ, Miranda E, Lomas DA.

FASEB J. 2015 Jun;29(6):2667-78. doi: 10.1096/fj.14-267351. Epub 2015 Mar 10.


Characterising the association of latency with α(1)-antitrypsin polymerisation using a novel monoclonal antibody.

Tan L, Perez J, Mela M, Miranda E, Burling KA, Rouhani FN, DeMeo DL, Haq I, Irving JA, Ordóñez A, Dickens JA, Brantly M, Marciniak SJ, Alexander GJ, Gooptu B, Lomas DA.

Int J Biochem Cell Biol. 2015 Jan;58:81-91. doi: 10.1016/j.biocel.2014.11.005. Epub 2014 Nov 13.


Pharmacokinetic bridging approach for developing biologics-delivery devices: a case study with a golimumab autoinjector.

Xu Z, Marciniak SJ Jr, Frederick B, Kim L, Zhuang Y, Davis HM, Zhou H.

Clin Ther. 2015 Feb 1;37(2):427-38. doi: 10.1016/j.clinthera.2014.09.012. Epub 2014 Oct 29.


Function of monocytes and monocyte-derived macrophages in α1-antitrypsin deficiency.

Van't Wout EF, van Schadewijk A, Lomas DA, Stolk J, Marciniak SJ, Hiemstra PS.

Eur Respir J. 2015 Feb;45(2):365-76. doi: 10.1183/09031936.00046114. Epub 2014 Oct 16.


Palliative treatment for symptomatic malignant pericardial effusion†.

Jama GM, Scarci M, Bowden J, Marciniak SJ.

Interact Cardiovasc Thorac Surg. 2014 Dec;19(6):1019-26. doi: 10.1093/icvts/ivu267. Epub 2014 Aug 20. Review.


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 SJ, Williams J, Lomas DA, Oliver SG, Favrin G, Sattelle DB, Crowther DC.

PLoS One. 2014 Jul 31;9(7):e102985. doi: 10.1371/journal.pone.0102985. eCollection 2014.


Cellular mechanisms of endoplasmic reticulum stress signaling in health and disease. 2. Protein misfolding and ER stress.

Chambers JE, Marciniak SJ.

Am J Physiol Cell Physiol. 2014 Oct 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. Review.


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. Epub 2014 Apr 28.


Guselkumab (an IL-23-specific mAb) demonstrates clinical and molecular response in patients with moderate-to-severe psoriasis.

Sofen H, Smith S, Matheson RT, Leonardi CL, Calderon C, Brodmerkel C, Li K, Campbell K, Marciniak SJ Jr, Wasfi Y, Wang Y, Szapary P, Krueger JG.

J Allergy Clin Immunol. 2014 Apr;133(4):1032-40. doi: 10.1016/j.jaci.2014.01.025.


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. Review.


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. Epub 2014 Mar 6. No abstract available.


Genetic susceptibility.

Marciniak SJ, Lomas DA.

Clin Chest Med. 2014 Mar;35(1):29-38. doi: 10.1016/j.ccm.2013.10.008. Epub 2013 Dec 12. Review.


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.

Hum Mol Genet. 2014 Feb 15;23(4):929-41. doi: 10.1093/hmg/ddt487. Epub 2013 Oct 4.


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.

Nature. 2013 Nov 14;503(7475):272-6. doi: 10.1038/nature12599. Epub 2013 Oct 2.


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 Dec;1832(12):2115-26. doi: 10.1016/j.bbadis.2013.07.019. Epub 2013 Aug 2.


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.

Hum Mol Genet. 2013 Nov 15;22(22):4616-26. doi: 10.1093/hmg/ddt310. Epub 2013 Jun 28.


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. Epub 2013 Feb 27.


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.

J Cell Sci. 2013 Mar 15;126(Pt 6):1406-15. doi: 10.1242/jcs.117614. Epub 2013 Feb 15.


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. Epub 2013 Feb 14.


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. Epub 2013 Jan 22.


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. Review.


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