Phagocytic glia are obligatory intermediates in transmission of mutant huntingtin aggregates across neuronal synapses.

Donnelly KM, DeLorenzo OR, Zaya AD, Pisano GE, Thu WM, Luo L, Kopito RR, Panning Pearce MM.Elife. 2020 May 28;9:e58499. doi: 10.7554/eLife.58499. Online ahead of print.PMID: 32463364 

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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 epub ahead of print

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.

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.

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.

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.

Prion-Like Characteristics of Polyglutamine-Containing Proteins.

Pearce MMP, Kopito RR.

Cold Spring Harb Perspect Med. 2018 Feb 1;8(2). pii: a024257.

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.

Protein misfolding in neurodegenerative diseases: implications and strategies.

Sweeney P, Park H, Baumann M, Dunlop J, Frydman J, Kopito R, McCampbell A, Leblanc G, Venkateswaran A, Nurmi A, Hodgson R.

Transl Neurodegener. 2017 Mar 13;6:6.

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.

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.

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

Kopito RR.

Trends Cell Biol. 2016 Aug;26(8):559-560.

Protein misfolding specifies recruitment to cytoplasmic inclusion bodies.

Bersuker K, Brandeis M, Kopito RR.

J Cell Biol. 2016 Apr 25;213(2):229-41.

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

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

Nat Commun. 2015 Apr 13;6:6768.

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.

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.

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.

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.