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Unique Structural Features of the Mitochondrial AAA+ Protease AFG3L2 Reveal the Molecular Basis for Activity in Health and Disease.

Puchades C, Ding B, Song A, Wiseman RL, Lander GC, Glynn SE.

Mol Cell. 2019 Sep 5;75(5):1073-1085.e6. doi: 10.1016/j.molcel.2019.06.016. Epub 2019 Jul 18.


Publisher Correction: Non-canonical function of IRE1α determines mitochondria-associated endoplasmic reticulum composition to control calcium transfer and bioenergetics.

Carreras-Sureda A, Jaña F, Urra H, Durand S, Mortenson DE, Sagredo A, Bustos G, Hazari Y, Ramos-Fernández E, Sassano ML, Pihán P, van Vliet AR, González-Quiroz M, Torres AK, Tapia-Rojas C, Kerkhofs M, Vicente R, Kaufman RJ, Inestrosa NC, Gonzalez-Billault C, Wiseman RL, Agostinis P, Bultynck G, Court FA, Kroemer G, Cárdenas JC, Hetz C.

Nat Cell Biol. 2019 Jul;21(7):913. doi: 10.1038/s41556-019-0355-9.


Non-canonical function of IRE1α determines mitochondria-associated endoplasmic reticulum composition to control calcium transfer and bioenergetics.

Carreras-Sureda A, Jaña F, Urra H, Durand S, Mortenson DE, Sagredo A, Bustos G, Hazari Y, Ramos-Fernández E, Sassano ML, Pihán P, van Vliet AR, González-Quiroz M, Torres AK, Tapia-Rojas C, Kerkhofs M, Vicente R, Kaufman RJ, Inestrosa NC, Gonzalez-Billault C, Wiseman RL, Agostinis P, Bultynck G, Court FA, Kroemer G, Cárdenas JC, Hetz C.

Nat Cell Biol. 2019 Jun;21(6):755-767. doi: 10.1038/s41556-019-0329-y. Epub 2019 May 20. Erratum in: Nat Cell Biol. 2019 Jun 14;:.


Quantitative Interactome Proteomics Reveals a Molecular Basis for ATF6-Dependent Regulation of a Destabilized Amyloidogenic Protein.

Plate L, Rius B, Nguyen B, Genereux JC, Kelly JW, Wiseman RL.

Cell Chem Biol. 2019 Jul 18;26(7):913-925.e4. doi: 10.1016/j.chembiol.2019.04.001. Epub 2019 May 16.


Proteostasis and Beyond: ATF6 in Ischemic Disease.

Glembotski CC, Rosarda JD, Wiseman RL.

Trends Mol Med. 2019 Jun;25(6):538-550. doi: 10.1016/j.molmed.2019.03.005. Epub 2019 May 8. Review.


Lining up for quality control: linear ubiquitin and proteotoxicity.

Wiseman RL.

EMBO J. 2019 May 2;38(9). pii: e101985. doi: 10.15252/embj.2019101985. Epub 2019 Apr 11. No abstract available.


Deconvoluting Stress-Responsive Proteostasis Signaling Pathways for Pharmacologic Activation Using Targeted RNA Sequencing.

Grandjean JMD, Plate L, Morimoto RI, Bollong MJ, Powers ET, Wiseman RL.

ACS Chem Biol. 2019 Apr 19;14(4):784-795. doi: 10.1021/acschembio.9b00134. Epub 2019 Mar 13.


PERK Signaling Regulates Extracellular Proteostasis of an Amyloidogenic Protein During Endoplasmic Reticulum Stress.

Romine IC, Wiseman RL.

Sci Rep. 2019 Jan 23;9(1):410. doi: 10.1038/s41598-018-37207-0.


Pharmacologic ATF6 activation confers global protection in widespread disease models by reprograming cellular proteostasis.

Blackwood EA, Azizi K, Thuerauf DJ, Paxman RJ, Plate L, Kelly JW, Wiseman RL, Glembotski CC.

Nat Commun. 2019 Jan 14;10(1):187. doi: 10.1038/s41467-018-08129-2.


Pharmacologic ATF6 activating compounds are metabolically activated to selectively modify endoplasmic reticulum proteins.

Paxman R, Plate L, Blackwood EA, Glembotski C, Powers ET, Wiseman RL, Kelly JW.

Elife. 2018 Aug 7;7. pii: e37168. doi: 10.7554/eLife.37168.


Coordinating Mitochondrial Biology Through the Stress-Responsive Regulation of Mitochondrial Proteases.

Lebeau J, Rainbolt TK, Wiseman RL.

Int Rev Cell Mol Biol. 2018;340:79-128. doi: 10.1016/bs.ircmb.2018.05.003. Epub 2018 Jun 22. Review.


A library of ATTR amyloidosis patient-specific induced pluripotent stem cells for disease modelling and in vitro testing of novel therapeutics.

Giadone RM, Rosarda JD, Akepati PR, Thomas AC, Boldbaatar B, James MF, Wilson AA, Sanchorawala V, Connors LH, Berk JL, Wiseman RL, Murphy GJ.

Amyloid. 2018 Sep;25(3):148-155. doi: 10.1080/13506129.2018.1489228. Epub 2018 Jul 21.


The PERK Arm of the Unfolded Protein Response Regulates Mitochondrial Morphology during Acute Endoplasmic Reticulum Stress.

Lebeau J, Saunders JM, Moraes VWR, Madhavan A, Madrazo N, Anthony MC, Wiseman RL.

Cell Rep. 2018 Mar 13;22(11):2827-2836. doi: 10.1016/j.celrep.2018.02.055.


Characterization of an A-Site Selective Protein Disulfide Isomerase A1 Inhibitor.

Cole KS, Grandjean JMD, Chen K, Witt CH, O'Day J, Shoulders MD, Wiseman RL, Weerapana E.

Biochemistry. 2018 Apr 3;57(13):2035-2043. doi: 10.1021/acs.biochem.8b00178. Epub 2018 Mar 19.


The unfolded protein response regulator ATF6 promotes mesodermal differentiation.

Kroeger H, Grimsey N, Paxman R, Chiang WC, Plate L, Jones Y, Shaw PX, Trejo J, Tsang SH, Powers E, Kelly JW, Wiseman RL, Lin JH.

Sci Signal. 2018 Feb 13;11(517). pii: eaan5785. doi: 10.1126/scisignal.aan5785.


Structure of the mitochondrial inner membrane AAA+ protease YME1 gives insight into substrate processing.

Puchades C, Rampello AJ, Shin M, Giuliano CJ, Wiseman RL, Glynn SE, Lander GC.

Science. 2017 Nov 3;358(6363). pii: eaao0464. doi: 10.1126/science.aao0464.


The endoplasmic reticulum HSP40 co-chaperone ERdj3/DNAJB11 assembles and functions as a tetramer.

Chen KC, Qu S, Chowdhury S, Noxon IC, Schonhoft JD, Plate L, Powers ET, Kelly JW, Lander GC, Wiseman RL.

EMBO J. 2017 Aug 1;36(15):2296-2309. doi: 10.15252/embj.201695616. Epub 2017 Jun 27.


Regulating Secretory Proteostasis through the Unfolded Protein Response: From Function to Therapy.

Plate L, Wiseman RL.

Trends Cell Biol. 2017 Oct;27(10):722-737. doi: 10.1016/j.tcb.2017.05.006. Epub 2017 Jun 21. Review.


Endoplasmic Reticulum Proteostasis Influences the Oligomeric State of an Amyloidogenic Protein Secreted from Mammalian Cells.

Chen JJ, Genereux JC, Suh EH, Vartabedian VF, Rius B, Qu S, Dendle MTA, Kelly JW, Wiseman RL.

Cell Chem Biol. 2016 Oct 20;23(10):1282-1293. doi: 10.1016/j.chembiol.2016.09.001. Epub 2016 Oct 6.


Small molecule proteostasis regulators that reprogram the ER to reduce extracellular protein aggregation.

Plate L, Cooley CB, Chen JJ, Paxman RJ, Gallagher CM, Madoux F, Genereux JC, Dobbs W, Garza D, Spicer TP, Scampavia L, Brown SJ, Rosen H, Powers ET, Walter P, Hodder P, Wiseman RL, Kelly JW.

Elife. 2016 Jul 20;5. pii: e15550. doi: 10.7554/eLife.15550.


Modulating protein quality control.

Plate L, Paxman RJ, Wiseman RL, Kelly JW.

Elife. 2016 Jul 20;5. pii: e18431. doi: 10.7554/eLife.18431.


Reciprocal Degradation of YME1L and OMA1 Adapts Mitochondrial Proteolytic Activity during Stress.

Rainbolt TK, Lebeau J, Puchades C, Wiseman RL.

Cell Rep. 2016 Mar 8;14(9):2041-2049. doi: 10.1016/j.celrep.2016.02.011. Epub 2016 Feb 25.


Chaperones in Neurodegeneration.

Lindberg I, Shorter J, Wiseman RL, Chiti F, Dickey CA, McLean PJ.

J Neurosci. 2015 Oct 14;35(41):13853-9. doi: 10.1523/JNEUROSCI.2600-15.2015. Review.


Targeting protein aggregation for the treatment of degenerative diseases.

Eisele YS, Monteiro C, Fearns C, Encalada SE, Wiseman RL, Powers ET, Kelly JW.

Nat Rev Drug Discov. 2015 Nov;14(11):759-80. doi: 10.1038/nrd4593. Epub 2015 Sep 4. Review.


Enhanced Aromatic Sequons Increase Oligosaccharyltransferase Glycosylation Efficiency and Glycan Homogeneity.

Murray AN, Chen W, Antonopoulos A, Hanson SR, Wiseman RL, Dell A, Haslam SM, Powers DL, Powers ET, Kelly JW.

Chem Biol. 2015 Aug 20;22(8):1052-62. doi: 10.1016/j.chembiol.2015.06.017. Epub 2015 Jul 16.


A fluorogenic aryl fluorosulfate for intraorganellar transthyretin imaging in living cells and in Caenorhabditis elegans.

Baranczak A, Liu Y, Connelly S, Du WG, Greiner ER, Genereux JC, Wiseman RL, Eisele YS, Bradbury NC, Dong J, Noodleman L, Sharpless KB, Wilson IA, Encalada SE, Kelly JW.

J Am Chem Soc. 2015 Jun 17;137(23):7404-14. doi: 10.1021/jacs.5b03042. Epub 2015 Jun 8.


Endoplasmic reticulum quality control and systemic amyloid disease: Impacting protein stability from the inside out.

Chen JJ, Genereux JC, Wiseman RL.

IUBMB Life. 2015 Jun;67(6):404-13. doi: 10.1002/iub.1386. Epub 2015 May 26. Review.


Regulating extracellular proteostasis capacity through the unfolded protein response.

Genereux JC, Wiseman RL.

Prion. 2015;9(1):10-21. doi: 10.1080/19336896.2015.1011887.


ATF6 activation reduces the secretion and extracellular aggregation of destabilized variants of an amyloidogenic protein.

Chen JJ, Genereux JC, Qu S, Hulleman JD, Shoulders MD, Wiseman RL.

Chem Biol. 2014 Nov 20;21(11):1564-74. doi: 10.1016/j.chembiol.2014.09.009. Epub 2014 Oct 23.


YME1L degradation reduces mitochondrial proteolytic capacity during oxidative stress.

Rainbolt TK, Saunders JM, Wiseman RL.

EMBO Rep. 2015 Jan;16(1):97-106. doi: 10.15252/embr.201438976. Epub 2014 Nov 27.


Unfolded protein response-induced ERdj3 secretion links ER stress to extracellular proteostasis.

Genereux JC, Qu S, Zhou M, Ryno LM, Wang S, Shoulders MD, Kaufman RJ, Lasmézas CI, Kelly JW, Wiseman RL.

EMBO J. 2015 Jan 2;34(1):4-19. doi: 10.15252/embj.201488896. Epub 2014 Oct 31.


Unfolded protein response activation reduces secretion and extracellular aggregation of amyloidogenic immunoglobulin light chain.

Cooley CB, Ryno LM, Plate L, Morgan GJ, Hulleman JD, Kelly JW, Wiseman RL.

Proc Natl Acad Sci U S A. 2014 Sep 9;111(36):13046-51. doi: 10.1073/pnas.1406050111. Epub 2014 Aug 25.


Stress-responsive regulation of mitochondria through the ER unfolded protein response.

Rainbolt TK, Saunders JM, Wiseman RL.

Trends Endocrinol Metab. 2014 Oct;25(10):528-37. doi: 10.1016/j.tem.2014.06.007. Epub 2014 Jul 18. Review.


Characterizing the altered cellular proteome induced by the stress-independent activation of heat shock factor 1.

Ryno LM, Genereux JC, Naito T, Morimoto RI, Powers ET, Shoulders MD, Wiseman RL.

ACS Chem Biol. 2014 Jun 20;9(6):1273-83. doi: 10.1021/cb500062n. Epub 2014 Apr 16.


Quantification of transthyretin kinetic stability in human plasma using subunit exchange.

Rappley I, Monteiro C, Novais M, Baranczak A, Solis G, Wiseman RL, Helmke S, Maurer MS, Coelho T, Powers ET, Kelly JW.

Biochemistry. 2014 Apr 1;53(12):1993-2006. doi: 10.1021/bi500171j. Epub 2014 Mar 24.


Stress-regulated translational attenuation adapts mitochondrial protein import through Tim17A degradation.

Rainbolt TK, Atanassova N, Genereux JC, Wiseman RL.

Cell Metab. 2013 Dec 3;18(6):908-19. doi: 10.1016/j.cmet.2013.11.006.


Induced pluripotent stem cell modeling of multisystemic, hereditary transthyretin amyloidosis.

Leung A, Nah SK, Reid W, Ebata A, Koch CM, Monti S, Genereux JC, Wiseman RL, Wolozin B, Connors LH, Berk JL, Seldin DC, Mostoslavsky G, Kotton DN, Murphy GJ.

Stem Cell Reports. 2013 Oct 31;1(5):451-63. doi: 10.1016/j.stemcr.2013.10.003. eCollection 2013.


Broadly applicable methodology for the rapid and dosable small molecule-mediated regulation of transcription factors in human cells.

Shoulders MD, Ryno LM, Cooley CB, Kelly JW, Wiseman RL.

J Am Chem Soc. 2013 Jun 5;135(22):8129-32. doi: 10.1021/ja402756p. Epub 2013 May 21.


Targeting unfolded protein response signaling pathways to ameliorate protein misfolding diseases.

Ryno LM, Wiseman RL, Kelly JW.

Curr Opin Chem Biol. 2013 Jun;17(3):346-52. doi: 10.1016/j.cbpa.2013.04.009. Epub 2013 May 4. Review.


Stress-independent activation of XBP1s and/or ATF6 reveals three functionally diverse ER proteostasis environments.

Shoulders MD, Ryno LM, Genereux JC, Moresco JJ, Tu PG, Wu C, Yates JR 3rd, Su AI, Kelly JW, Wiseman RL.

Cell Rep. 2013 Apr 25;3(4):1279-92. doi: 10.1016/j.celrep.2013.03.024. Epub 2013 Apr 11.


Hypoxemia in healthy subjects at moderate altitude.

Wiseman RL, Kelly PT, Swanney MP, McNamara KP, Beckert L.

Aviat Space Environ Med. 2013 Jan;84(1):22-6.


Tafamidis, a potent and selective transthyretin kinetic stabilizer that inhibits the amyloid cascade.

Bulawa CE, Connelly S, Devit M, Wang L, Weigel C, Fleming JA, Packman J, Powers ET, Wiseman RL, Foss TR, Wilson IA, Kelly JW, Labaudinière R.

Proc Natl Acad Sci U S A. 2012 Jun 12;109(24):9629-34. doi: 10.1073/pnas.1121005109. Epub 2012 May 29.


Lack of evidence for a link between latanoprost use and malignant melanoma: an analysis of safety databases and a review of the literature.

Tressler CS, Wiseman RL, Dombi TM, Jessen B, Huang K, Kwok KK, Wirostko BM.

Br J Ophthalmol. 2011 Nov;95(11):1490-5. doi: 10.1136/bjo.2010.193987. Epub 2011 Apr 21. Review.


Cell biology. Phosphatase inhibition delays translational recovery.

Wiseman RL, Kelly JW.

Science. 2011 Apr 1;332(6025):44-5. doi: 10.1126/science.1204505. No abstract available.


Chemically targeting the emergent properties of a chaperone complex.

Genereux JC, Wiseman RL.

Chem Biol. 2011 Feb 25;18(2):144-5. doi: 10.1016/j.chembiol.2011.02.002.


Flavonol activation defines an unanticipated ligand-binding site in the kinase-RNase domain of IRE1.

Wiseman RL, Zhang Y, Lee KP, Harding HP, Haynes CM, Price J, Sicheri F, Ron D.

Mol Cell. 2010 Apr 23;38(2):291-304. doi: 10.1016/j.molcel.2010.04.001.


SnapShot: The unfolded protein response.

Wiseman RL, Haynes CM, Ron D.

Cell. 2010 Feb 19;140(4):590-590.e2. doi: 10.1016/j.cell.2010.02.006. No abstract available.


Evolving protein stability through genetic selection.

Wiseman RL, Kelly JW.

Mol Cell. 2009 Dec 11;36(5):730-1. doi: 10.1016/j.molcel.2009.11.023.


Thioredoxin-related Protein 32 is an arsenite-regulated Thiol Reductase of the proteasome 19 S particle.

Wiseman RL, Chin KT, Haynes CM, Stanhill A, Xu CF, Roguev A, Krogan NJ, Neubert TA, Ron D.

J Biol Chem. 2009 May 29;284(22):15233-45. doi: 10.1074/jbc.M109.002121. Epub 2009 Apr 6.


An adaptable standard for protein export from the endoplasmic reticulum.

Wiseman RL, Powers ET, Buxbaum JN, Kelly JW, Balch WE.

Cell. 2007 Nov 16;131(4):809-21.

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