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Acute unfolding of a single protein immediately stimulates recruitment of ubiquitin protein ligase E3C (UBE3C) to 26S proteasomes.

Gottlieb CD, Thompson ACS, Ordureau A, Harper JW, Kopito RR.

J Biol Chem. 2019 Nov 8;294(45):16511-16524. doi: 10.1074/jbc.RA119.009654. Epub 2019 Aug 2.


Methods for genetic analysis of mammalian ER-associated degradation.

Leto DE, Kopito RR.

Methods Enzymol. 2019;619:97-120. doi: 10.1016/bs.mie.2019.01.006. Epub 2019 Feb 20.


Ribosomal protein RPL26 is the principal target of UFMylation.

Walczak CP, Leto DE, Zhang L, Riepe C, Muller RY, DaRosa PA, Ingolia NT, Elias JE, Kopito RR.

Proc Natl Acad Sci U S A. 2019 Jan 22;116(4):1299-1308. doi: 10.1073/pnas.1816202116. Epub 2019 Jan 9.


Genome-wide CRISPR Analysis Identifies Substrate-Specific Conjugation Modules in ER-Associated Degradation.

Leto DE, Morgens DW, Zhang L, Walczak CP, Elias JE, Bassik MC, Kopito RR.

Mol Cell. 2019 Jan 17;73(2):377-389.e11. doi: 10.1016/j.molcel.2018.11.015. Epub 2018 Dec 20.


Proteomic analysis of monolayer-integrated proteins on lipid droplets identifies amphipathic interfacial α-helical membrane anchors.

Pataki CI, Rodrigues J, Zhang L, Qian J, Efron B, Hastie T, Elias JE, Levitt M, Kopito RR.

Proc Natl Acad Sci U S A. 2018 Aug 28;115(35):E8172-E8180. doi: 10.1073/pnas.1807981115. Epub 2018 Aug 13.


Redundant and Antagonistic Roles of XTP3B and OS9 in Decoding Glycan and Non-glycan Degrons in ER-Associated Degradation.

van der Goot AT, Pearce MMP, Leto DE, Shaler TA, Kopito RR.

Mol Cell. 2018 May 3;70(3):516-530.e6. doi: 10.1016/j.molcel.2018.03.026. Epub 2018 Apr 26.


Characterization of protein complexes of the endoplasmic reticulum-associated degradation E3 ubiquitin ligase Hrd1.

Hwang J, Walczak CP, Shaler TA, Olzmann JA, Zhang L, Elias JE, Kopito RR.

J Biol Chem. 2017 Jun 2;292(22):9104-9116. doi: 10.1074/jbc.M117.785055. Epub 2017 Apr 14.


Prion-Like Characteristics of Polyglutamine-Containing Proteins.

Pearce MMP, Kopito RR.

Cold Spring Harb Perspect Med. 2018 Feb 1;8(2). pii: a024257. doi: 10.1101/cshperspect.a024257. Review.


Ubiquitin Accumulation on Disease Associated Protein Aggregates Is Correlated with Nuclear Ubiquitin Depletion, Histone De-Ubiquitination and Impaired DNA Damage Response.

Ben Yehuda A, Risheq M, Novoplansky O, Bersuker K, Kopito RR, Goldberg M, Brandeis M.

PLoS One. 2017 Jan 4;12(1):e0169054. doi: 10.1371/journal.pone.0169054. eCollection 2017.


Peroxin-dependent targeting of a lipid-droplet-destined membrane protein to ER subdomains.

Schrul B, Kopito RR.

Nat Cell Biol. 2016 Jul;18(7):740-51. doi: 10.1038/ncb3373. Epub 2016 Jun 13.


Ron R. Kopito: Unfolding the Secrets of Protein Aggregation.

Kopito RR.

Trends Cell Biol. 2016 Aug;26(8):559-560. doi: 10.1016/j.tcb.2016.05.001. Epub 2016 May 27. No abstract available.


Protein misfolding specifies recruitment to cytoplasmic inclusion bodies.

Bersuker K, Brandeis M, Kopito RR.

J Cell Biol. 2016 Apr 25;213(2):229-41. doi: 10.1083/jcb.201511024.


Prion-like transmission of neuronal huntingtin aggregates to phagocytic glia in the Drosophila brain.

Pearce MMP, Spartz EJ, Hong W, Luo L, Kopito RR.

Nat Commun. 2015 Apr 13;6:6768. doi: 10.1038/ncomms7768.


Heat shock response activation exacerbates inclusion body formation in a cellular model of Huntington disease.

Bersuker K, Hipp MS, Calamini B, Morimoto RI, Kopito RR.

J Biol Chem. 2013 Aug 16;288(33):23633-8. doi: 10.1074/jbc.C113.481945. Epub 2013 Jul 9.


Simultaneous measurement of amyloid fibril formation by dynamic light scattering and fluorescence reveals complex aggregation kinetics.

Streets AM, Sourigues Y, Kopito RR, Melki R, Quake SR.

PLoS One. 2013;8(1):e54541. doi: 10.1371/journal.pone.0054541. Epub 2013 Jan 17.


Spatial regulation of UBXD8 and p97/VCP controls ATGL-mediated lipid droplet turnover.

Olzmann JA, Richter CM, Kopito RR.

Proc Natl Acad Sci U S A. 2013 Jan 22;110(4):1345-50. doi: 10.1073/pnas.1213738110. Epub 2013 Jan 7.


The mammalian endoplasmic reticulum-associated degradation system.

Olzmann JA, Kopito RR, Christianson JC.

Cold Spring Harb Perspect Biol. 2013 Sep 1;5(9). pii: a013185. doi: 10.1101/cshperspect.a013185. Review.


ALIX is a Lys63-specific polyubiquitin binding protein that functions in retrovirus budding.

Dowlatshahi DP, Sandrin V, Vivona S, Shaler TA, Kaiser SE, Melandri F, Sundquist WI, Kopito RR.

Dev Cell. 2012 Dec 11;23(6):1247-54. doi: 10.1016/j.devcel.2012.10.023. Epub 2012 Nov 29.


Unassembled CD147 is an endogenous endoplasmic reticulum-associated degradation substrate.

Tyler RE, Pearce MM, Shaler TA, Olzmann JA, Greenblatt EJ, Kopito RR.

Mol Biol Cell. 2012 Dec;23(24):4668-78. doi: 10.1091/mbc.E12-06-0428. Epub 2012 Oct 24.


Making the cut: intramembrane cleavage by a rhomboid protease promotes ERAD.

Greenblatt EJ, Olzmann JA, Kopito RR.

Nat Struct Mol Biol. 2012 Oct;19(10):979-81. doi: 10.1038/nsmb.2398.


Fibrillar structure and charge determine the interaction of polyglutamine protein aggregates with the cell surface.

Trevino RS, Lauckner JE, Sourigues Y, Pearce MM, Bousset L, Melki R, Kopito RR.

J Biol Chem. 2012 Aug 24;287(35):29722-8. doi: 10.1074/jbc.M112.372474. Epub 2012 Jun 29.


Perturbation of the hematopoietic system during embryonic liver development due to disruption of polyubiquitin gene Ubc in mice.

Ryu KY, Park H, Rossi DJ, Weissman IL, Kopito RR.

PLoS One. 2012;7(2):e32956. doi: 10.1371/journal.pone.0032956. Epub 2012 Feb 29.


Indirect inhibition of 26S proteasome activity in a cellular model of Huntington's disease.

Hipp MS, Patel CN, Bersuker K, Riley BE, Kaiser SE, Shaler TA, Brandeis M, Kopito RR.

J Cell Biol. 2012 Mar 5;196(5):573-87. doi: 10.1083/jcb.201110093. Epub 2012 Feb 27.


Live-cell imaging of ubiquitin-proteasome system function.

Hipp MS, Bersuker K, Kopito RR.

Methods Mol Biol. 2012;832:463-72. doi: 10.1007/978-1-61779-474-2_33.


Defining human ERAD networks through an integrative mapping strategy.

Christianson JC, Olzmann JA, Shaler TA, Sowa ME, Bennett EJ, Richter CM, Tyler RE, Greenblatt EJ, Harper JW, Kopito RR.

Nat Cell Biol. 2011 Nov 27;14(1):93-105. doi: 10.1038/ncb2383.


Derlin-1 is a rhomboid pseudoprotease required for the dislocation of mutant α-1 antitrypsin from the endoplasmic reticulum.

Greenblatt EJ, Olzmann JA, Kopito RR.

Nat Struct Mol Biol. 2011 Sep 11;18(10):1147-52. doi: 10.1038/nsmb.2111.


Protein standard absolute quantification (PSAQ) method for the measurement of cellular ubiquitin pools.

Kaiser SE, Riley BE, Shaler TA, Trevino RS, Becker CH, Schulman H, Kopito RR.

Nat Methods. 2011 Jul 10;8(8):691-6. doi: 10.1038/nmeth.1649.


Lipid droplet formation is dispensable for endoplasmic reticulum-associated degradation.

Olzmann JA, Kopito RR.

J Biol Chem. 2011 Aug 12;286(32):27872-4. doi: 10.1074/jbc.C111.266452. Epub 2011 Jun 21.


Altered testicular gene expression patterns in mice lacking the polyubiquitin gene Ubb.

Sinnar SA, Small CL, Evanoff RM, Reinholdt LG, Griswold MD, Kopito RR, Ryu KY.

Mol Reprod Dev. 2011 Jun;78(6):415-25. doi: 10.1002/mrd.21318. Epub 2011 May 3.


Autophagy inhibition engages Nrf2-p62 Ub-associated signaling.

Riley BE, Kaiser SE, Kopito RR.

Autophagy. 2011 Mar;7(3):338-40. No abstract available.


Ubiquitin accumulation in autophagy-deficient mice is dependent on the Nrf2-mediated stress response pathway: a potential role for protein aggregation in autophagic substrate selection.

Riley BE, Kaiser SE, Shaler TA, Ng AC, Hara T, Hipp MS, Lage K, Xavier RJ, Ryu KY, Taguchi K, Yamamoto M, Tanaka K, Mizushima N, Komatsu M, Kopito RR.

J Cell Biol. 2010 Nov 1;191(3):537-52. doi: 10.1083/jcb.201005012.


Loss of polyubiquitin gene Ubb leads to metabolic and sleep abnormalities in mice.

Ryu KY, Fujiki N, Kazantzis M, Garza JC, Bouley DM, Stahl A, Lu XY, Nishino S, Kopito RR.

Neuropathol Appl Neurobiol. 2010 Jun;36(4):285-99. doi: 10.1111/j.1365-2990.2009.01057.x. Epub 2009 Dec 8.


SPFH1 and SPFH2 mediate the ubiquitination and degradation of inositol 1,4,5-trisphosphate receptors in muscarinic receptor-expressing HeLa cells.

Wang Y, Pearce MM, Sliter DA, Olzmann JA, Christianson JC, Kopito RR, Boeckmann S, Gagen C, Leichner GS, Roitelman J, Wojcikiewicz RJ.

Biochim Biophys Acta. 2009 Nov;1793(11):1710-8. doi: 10.1016/j.bbamcr.2009.09.004. Epub 2009 Sep 12.


The polyubiquitin Ubc gene modulates histone H2A monoubiquitylation in the R6/2 mouse model of Huntington's disease.

Bett JS, Benn CL, Ryu KY, Kopito RR, Bates GP.

J Cell Mol Med. 2009 Aug;13(8B):2645-57. doi: 10.1111/j.1582-4934.2008.00543.x.


Cytoplasmic penetration and persistent infection of mammalian cells by polyglutamine aggregates.

Ren PH, Lauckner JE, Kachirskaia I, Heuser JE, Melki R, Kopito RR.

Nat Cell Biol. 2009 Feb;11(2):219-25. doi: 10.1038/ncb1830. Epub 2009 Jan 18.


Hypothalamic neurodegeneration and adult-onset obesity in mice lacking the Ubb polyubiquitin gene.

Ryu KY, Garza JC, Lu XY, Barsh GS, Kopito RR.

Proc Natl Acad Sci U S A. 2008 Mar 11;105(10):4016-21. doi: 10.1073/pnas.0800096105. Epub 2008 Feb 25.


OS-9 and GRP94 deliver mutant alpha1-antitrypsin to the Hrd1-SEL1L ubiquitin ligase complex for ERAD.

Christianson JC, Shaler TA, Tyler RE, Kopito RR.

Nat Cell Biol. 2008 Mar;10(3):272-82. doi: 10.1038/ncb1689. Epub 2008 Feb 10.


The mouse polyubiquitin gene Ubb is essential for meiotic progression.

Ryu KY, Sinnar SA, Reinholdt LG, Vaccari S, Hall S, Garcia MA, Zaitseva TS, Bouley DM, Boekelheide K, Handel MA, Conti M, Kopito RR.

Mol Cell Biol. 2008 Feb;28(3):1136-46. Epub 2007 Dec 10.


Global changes to the ubiquitin system in Huntington's disease.

Bennett EJ, Shaler TA, Woodman B, Ryu KY, Zaitseva TS, Becker CH, Bates GP, Schulman H, Kopito RR.

Nature. 2007 Aug 9;448(7154):704-8.


The mouse polyubiquitin gene UbC is essential for fetal liver development, cell-cycle progression and stress tolerance.

Ryu KY, Maehr R, Gilchrist CA, Long MA, Bouley DM, Mueller B, Ploegh HL, Kopito RR.

EMBO J. 2007 Jun 6;26(11):2693-706. Epub 2007 May 10.


[Cellular mechanisms of protein quality control].

Bennett EJ, Shaler T, Gonzalez-Zulueta M, Schulman HF, Iwata A, Riley BE, Johnston JA, Bucci M, Nukina N, Ellerby L, Kopito RR.

Rinsho Shinkeigaku. 2006 Nov;46(11):805. Japanese. No abstract available.


Impaired post-translational folding of familial ALS-linked Cu, Zn superoxide dismutase mutants.

Bruns CK, Kopito RR.

EMBO J. 2007 Feb 7;26(3):855-66. Epub 2007 Jan 25.


Central pore residues mediate the p97/VCP activity required for ERAD.

DeLaBarre B, Christianson JC, Kopito RR, Brunger AT.

Mol Cell. 2006 May 19;22(4):451-62.


Ubiquitin-specific protease 2 as a tool for quantification of total ubiquitin levels in biological specimens.

Ryu KY, Baker RT, Kopito RR.

Anal Biochem. 2006 Jun 1;353(1):153-5. Epub 2006 Apr 18. No abstract available.


Application and analysis of the GFPu family of ubiquitin-proteasome system reporters.

Bence NF, Bennett EJ, Kopito RR.

Methods Enzymol. 2005;399:481-90.


HDAC6 and microtubules are required for autophagic degradation of aggregated huntingtin.

Iwata A, Riley BE, Johnston JA, Kopito RR.

J Biol Chem. 2005 Dec 2;280(48):40282-92. Epub 2005 Sep 28.


Increased susceptibility of cytoplasmic over nuclear polyglutamine aggregates to autophagic degradation.

Iwata A, Christianson JC, Bucci M, Ellerby LM, Nukina N, Forno LS, Kopito RR.

Proc Natl Acad Sci U S A. 2005 Sep 13;102(37):13135-40. Epub 2005 Sep 2.


Formation of morphologically similar globular aggregates from diverse aggregation-prone proteins in mammalian cells.

Mukai H, Isagawa T, Goyama E, Tanaka S, Bence NF, Tamura A, Ono Y, Kopito RR.

Proc Natl Acad Sci U S A. 2005 Aug 2;102(31):10887-92. Epub 2005 Jul 22.


Subversion of cellular autophagosomal machinery by RNA viruses.

Jackson WT, Giddings TH Jr, Taylor MP, Mulinyawe S, Rabinovitch M, Kopito RR, Kirkegaard K.

PLoS Biol. 2005 May;3(5):e156. Epub 2005 Apr 26.


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