Search Results - "Kopito, Ron R"

Spatial regulation of UBXD8 and p97/VCP controls ATGL-mediated lipid droplet turnover by Olzmann, James A., Richter, Caleb M., Kopito, Ron R.

“…UBXD8 is a membrane-embedded recruitment factor for the p97/VCP segregase that has been previously linked to endoplasmic reticulum (ER)-associated degradation…”
Get full text

RPL26/uL24 UFMylation is essential for ribosome-associated quality control at the endoplasmic reticulum by Scavone, Francesco, Gumbin, Samantha C, Da Rosa, Paul A, Kopito, Ron R

“…Ribosomes that stall while translating cytosolic proteins are incapacitated by incomplete nascent chains, termed "arrest peptides" (APs) that are destroyed by…”
Get full text

Ribosomal protein RPL26 is the principal target of UFMylation by Walczak, Christopher P., Leto, Dara E., Zhang, Lichao, Riepe, Celeste, Muller, Ryan Y., DaRosa, Paul A., Ingolia, Nicholas T., Elias, Joshua E., Kopito, Ron R.

“…Ubiquitin fold modifier 1 (UFM1) is a small, metazoan-specific, ubiquitin-like protein modifier that is essential for embryonic development. Although…”
Get full text

Prion-like transmission of neuronal huntingtin aggregates to phagocytic glia in the Drosophila brain by Pearce, Margaret M. P., Spartz, Ellen J., Hong, Weizhe, Luo, Liqun, Kopito, Ron R.

“…The brain has a limited capacity to self-protect against protein aggregate-associated pathology, and mounting evidence supports a role for phagocytic glia in…”
Get full text

Defining human ERAD networks through an integrative mapping strategy by Christianson, John C., Olzmann, James A., Shaler, Thomas A., Sowa, Mathew E., Bennett, Eric J., Richter, Caleb M., Tyler, Ryan E., Greenblatt, Ethan J., Wade Harper, J., Kopito, Ron R.

“…Proteins that fail to correctly fold or assemble into oligomeric complexes in the endoplasmic reticulum (ER) are degraded by a ubiquitin- and…”
Get full text

Protein misfolding specifies recruitment to cytoplasmic inclusion bodies by Bersuker, Kirill, Brandeis, Michael, Kopito, Ron R

“…Inclusion bodies (IBs) containing aggregated disease-associated proteins and polyubiquitin (poly-Ub) conjugates are universal histopathological features of…”
Get full text

Cytoplasmic penetration and persistent infection of mammalian cells by polyglutamine aggregates by Kachirskaia, Ioulia, Heuser, John E, Ren, Pei-Hsien, Melki, Ronald, Kopito, Ron R, Lauckner, Jane E

“…Sequence-specific nucleated protein aggregation is closely linked to the pathogenesis of most neurodegenerative diseases and constitutes the molecular basis of…”
Get full text

Redundant and Antagonistic Roles of XTP3B and OS9 in Decoding Glycan and Non-glycan Degrons in ER-Associated Degradation by van der Goot, Annemieke T., Pearce, Margaret M.P., Leto, Dara E., Shaler, Thomas A., Kopito, Ron R.

“…Glycoproteins engaged in unproductive folding in the ER are marked for degradation by a signal generated by progressive demannosylation of substrate N-glycans…”
Get full text

Prion-Like Characteristics of Polyglutamine-Containing Proteins by Pearce, Margaret M P, Kopito, Ron R

“…Transmissible spongiform encephalopathies are infectious neurodegenerative diseases caused by the conversion of prion protein (PrP) into a self-replicating…”
Get full text

Indirect inhibition of 26S proteasome activity in a cellular model of Huntington's disease by Hipp, Mark S, Patel, Chetan N, Bersuker, Kirill, Riley, Brigit E, Kaiser, Stephen E, Shaler, Thomas A, Brandeis, Michael, Kopito, Ron R

“…Pathognomonic accumulation of ubiquitin (Ub) conjugates in human neurodegenerative diseases, such as Huntington's disease, suggests that highly aggregated…”
Get full text

Simultaneous measurement of amyloid fibril formation by dynamic light scattering and fluorescence reveals complex aggregation kinetics by Streets, Aaron M, Sourigues, Yannick, Kopito, Ron R, Melki, Ronald, Quake, Stephen R

“…An apparatus that combines dynamic light scattering and Thioflavin T fluorescence detection is used to simultaneously probe fibril formation in polyglutamine…”
Get full text

HDAC6 and Microtubules Are Required for Autophagic Degradation of Aggregated Huntingtin by Iwata, Atsushi, Riley, Brigit E., Johnston, Jennifer A., Kopito, Ron R.

“…CNS neurons are endowed with the ability to recover from cytotoxic insults associated with the accumulation of proteinaceous polyglutamine aggregates via a…”
Get full text

Global changes to the ubiquitin system in Huntington's disease by Kopito, Ron R, Bennett, Eric J, Shaler, Thomas A, Woodman, Ben, Ryu, Kwon-Yul, Zaitseva, Tatiana S, Becker, Christopher H, Bates, Gillian P, Schulman, Howard

“…Huntington's disease (HD) is a dominantly inherited neurodegenerative disorder caused by expansion of CAG triplet repeats in the huntingtin (HTT) gene (also…”
Get full text

Subversion of cellular autophagosomal machinery by RNA viruses by Jackson, William T, Giddings, Jr, Thomas H, Taylor, Matthew P, Mulinyawe, Sara, Rabinovitch, Marlene, Kopito, Ron R, Kirkegaard, Karla

“…Infection of human cells with poliovirus induces the proliferation of double-membraned cytoplasmic vesicles whose surfaces are used as the sites of viral RNA…”
Get full text

Lipid Droplet Formation Is Dispensable for Endoplasmic Reticulum-associated Degradation by Olzmann, James A., Kopito, Ron R.

“…Proteins that fail to fold or assemble in the endoplasmic reticulum (ER) are destroyed by cytoplasmic proteasomes through a process known as ER-associated…”
Get full text

Unassembled CD147 is an endogenous endoplasmic reticulum-associated degradation substrate by Tyler, Ryan E, Pearce, Margaret M P, Shaler, Thomas A, Olzmann, James A, Greenblatt, Ethan J, Kopito, Ron R

“…Degradation of folding- or assembly-defective proteins by the endoplasmic reticulum-associated degradation (ERAD) ubiquitin ligase, Hrd1, is facilitated by a…”
Get full text

Global Impairment of the Ubiquitin-Proteasome System by Nuclear or Cytoplasmic Protein Aggregates Precedes Inclusion Body Formation by Bennett, Eric J., Bence, Neil F., Jayakumar, Rajadas, Kopito, Ron R.

“…The highly conserved ubiquitin-proteasome system (UPS) controls the stability of most nuclear and cytoplasmic proteins and is therefore essential for virtually…”
Get full text

Ubiquitin Accumulation on Disease Associated Protein Aggregates Is Correlated with Nuclear Ubiquitin Depletion, Histone De-Ubiquitination and Impaired DNA Damage Response by Ben Yehuda, Adi, Risheq, Marwa, Novoplansky, Ofra, Bersuker, Kirill, Kopito, Ron R, Goldberg, Michal, Brandeis, Michael

“…Deposition of ubiquitin conjugates on inclusion bodies composed of protein aggregates is a definitive cytopathological hallmark of neurodegenerative diseases…”
Get full text

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 by Riley, Brigit E, Kaiser, Stephen E, Shaler, Thomas A, Ng, Aylwin C.Y, Hara, Taichi, Hipp, Mark S, Lage, Kasper, Xavier, Ramnik J, Ryu, Kwon-Yul, Taguchi, Keiko, Yamamoto, Masayuki, Tanaka, Keiji, Mizushima, Noboru, Komatsu, Masaaki, Kopito, Ron R

“…Genetic ablation of autophagy in mice leads to liver and brain degeneration accompanied by the appearance of ubiquitin (Ub) inclusions, which has been…”
Get full text

ALIX Is a Lys63-Specific Polyubiquitin Binding Protein that Functions in Retrovirus Budding by Dowlatshahi, Dara P., Sandrin, Virginie, Vivona, Sandro, Shaler, Thomas A., Kaiser, Stephen E., Melandri, Francesco, Sundquist, Wesley I., Kopito, Ron R.

“…The diversity of ubiquitin (Ub)-dependent signaling is attributed to the ability of this small protein to form different types of covalently linked polyUb…”
Get full text