Search Results - "Kopito, RR"

Aggresomes, inclusion bodies and protein aggregation by Kopito, Ron R

“…Intracellular and extracellular accumulation of aggregated protein are linked to many diseases, including ageing-related neurodegeneration and systemic…”
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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…”
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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…”
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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…”
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Impairment of the Ubiquitin-Proteasome System by Protein Aggregation by Bence, Neil F., Sampat, Roopal M., Kopito, Ron R.

“…Intracellular deposition of aggregated and ubiquitylated proteins is a prominent cytopathological feature of most neurodegenerative disorders. Whether protein…”
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Increased Susceptibility of Cytoplasmic over Nuclear Polyglutamine Aggregates to Autophagic Degradation by Iwata, Atsushi, Christianson, John C., Bucci, Mirella, Ellerby, Lisa M., Nukina, Nobuyuki, Forno, Lysia S., Kopito, Ron R., Farquhar, Marilyn Gist

“…CNS neurons are endowed with the ability to recover from cytotoxic insults associated with the accumulation of proteinaceous aggregates in mouse models of…”
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Conformational disease by Kopito, Ron R, Ron, David

“…A large and diverse number of diseases are now recognized as 'conformational diseases', caused by adoption of non-native protein conformations that lead to…”
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ER Quality Control: The Cytoplasmic Connection by Kopito, Ron R

“…The endoplasmic reticulum (ER) is the port of entry of membrane and secretory proteins into the central vacuolar system, which includes the ER itself, the…”
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Aggresomes: A Cellular Response to Misfolded Proteins by Johnston, Jennifer A., Ward, Cristina L., Kopito, Ron R.

“…Intracellular deposition of misfolded protein aggregates into ubiquitin-rich cytoplasmic inclusions is linked to the pathogenesis of many diseases. Why these…”
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Formation of High Molecular Weight Complexes of Mutant Cu,Zn-Superoxide Dismutase in a Mouse Model for Familial Amyotrophic Lateral Sclerosis by Johnston, Jennifer A., Dalton, Muffie J., Gurney, Mark E., Kopito, Ron R.

“…Deposition of aggregated protein into neurofilament-rich cytoplasmic inclusion bodies is a common cytopathological feature of neurodegenerative disease…”
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Aggresomes and Russell bodies. Symptoms of cellular indigestion? by Kopito, R R, Sitia, R

“…All cells are equipped with a proteolytic apparatus that eliminates damaged, misfolded and incorrectly assembled proteins. The principal engine of cytoplasmic…”
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A Rhodopsin Mutant Linked to Autosomal Dominant Retinitis Pigmentosa Is Prone to Aggregate and Interacts with the Ubiquitin Proteasome System by Illing, Michelle E., Rajan, Rahul S., Bence, Neil F., Kopito, Ron R.

“…The inherited retinal degenerations are typified by retinitis pigmentosa (RP), a heterogeneous group of inherited disorders that causes the destruction of…”
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Degradation of CFTR by the ubiquitin-proteasome pathway by Ward, Cristina L., Omura, Satoshi, Kopito, Ron R.

“…Most cases of cystic fibrosis are caused by mutations that interfere with the biosynthetic folding of the cystic fibrosis transmembrane conductance regulator…”
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Specificity in Intracellular Protein Aggregation and Inclusion Body Formation by Rajan, Rahul S., Illing, Michelle E., Bence, Neil F., Kopito, Ron R.

“…Protein aggregation is widely considered to be a nonspecific coalescence of misfolded proteins, driven by interactions between solvent-exposed hydrophobic…”
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The role of multiubiquitination in dislocation and degradation of the alpha subunit of the T cell antigen receptor by Yu, H, Kopito, R R

“…Unassembled alpha subunits of the T cell receptor (TCRalpha) are degraded by proteasomes following their dislocation from the endoplasmic reticulum membrane…”
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A Principal Role for the Proteasome in Endoplasmic Reticulum-associated Degradation of Misfolded Intracellular Cystic Fibrosis Transmembrane Conductance Regulator by Gelman, Marina S., Kannegaard, Elisa S., Kopito, Ron R.

“…Endoplasmic reticulum-associated degradation of misfolded cystic fibrosis transmembrane conductance regulator (CFTR) protein is known to involve the…”
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Suppression of Wild-type Rhodopsin Maturation by Mutants Linked to Autosomal Dominant Retinitis Pigmentosa by Rajan, Rahul S., Kopito, Ron R.

“…Autosomal dominant retinitis pigmentosa (ADRP) has been linked to mutations in the gene encoding rhodopsin. Most RP-linked rhodopsin mutants are unable to fold…”
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Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins by Ward, C L, Kopito, R R

“…Mutant (delta F508) and wild-type cystic fibrosis transmembrane conductance regulator (CFTR) were synthesized initially as an approximately 140-kDa…”
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Cytosolic Degradation of T-cell Receptor α Chains by the Proteasome by Yu, Helen, Kaung, Geoffrey, Kobayashi, Sumire, Kopito, Ron R.

“…The T-cell antigen receptor (TCR) is an hetero-oligomeric membrane complex composed of at least seven transmembrane polypeptide chains that has served as a…”
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Formation of Morphologically Similar Globular Aggregates from Diverse Aggregation-Prone Proteins in Mammalian Cells by Mukai, Hideyuki, Isagawa, Takayuki, Goyama, Emiko, Tanaka, Shuhei, Bence, Neil F., Tamura, Atsuo, Ono, Yoshitaka, Kopito, Ron R., Ingram, Vernon Martin

“…Huntington's disease is a progressive neurodegenerative disorder caused by a polyglutamine repeat expansion in the first exon of the huntingtin (Htt) protein…”
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