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R R Kopito

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Articles 58
Citations 5038
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Recent Articles
1.
Ryu K, Fujiki N, Kazantzis M, Garza J, Bouley D, Stahl A, et al.
Neuropathol Appl Neurobiol . 2009 Dec; 36(4):285-99. PMID: 20002312
Aims: Ubiquitin performs essential roles in a myriad of signalling pathways required for cellular function and survival. Recently, we reported that disruption of the stress-inducible ubiquitin-encoding gene Ubb reduces ubiquitin...
2.
Gilchrist C, Gray D, Stieber A, Gonatas N, Kopito R
Neuropathol Appl Neurobiol . 2005 Jan; 31(1):20-33. PMID: 15634228
The ubiquitin-proteasome system (UPS) is a central component in the cellular defence against potentially toxic protein aggregates. UPS dysfunction is linked to the pathogenesis of both sporadic and inherited neurodegenerative...
3.
Rajan R, Illing M, Bence N, Kopito R
Proc Natl Acad Sci U S A . 2001 Nov; 98(23):13060-5. PMID: 11687604
Protein aggregation is widely considered to be a nonspecific coalescence of misfolded proteins, driven by interactions between solvent-exposed hydrophobic surfaces that are normally buried within a protein's interior. Accordingly, abnormal...
4.
Bence N, Sampat R, Kopito R
Science . 2001 May; 292(5521):1552-5. PMID: 11375494
Intracellular deposition of aggregated and ubiquitylated proteins is a prominent cytopathological feature of most neurodegenerative disorders. Whether protein aggregates themselves are pathogenic or are the consequence of an underlying molecular...
5.
Kopito R, Sitia R
EMBO Rep . 2001 Mar; 1(3):225-31. PMID: 11256604
All cells are equipped with a proteolytic apparatus that eliminates damaged, misfolded and incorrectly assembled proteins. The principal engine of cytoplasmic proteolysis, the 26S proteasome, requires that substrates be unfolded...
6.
Kopito R
Trends Cell Biol . 2000 Dec; 10(12):524-30. PMID: 11121744
Intracellular and extracellular accumulation of aggregated protein are linked to many diseases, including ageing-related neurodegeneration and systemic amyloidosis. Cells avoid accumulating potentially toxic aggregates by mechanisms including the suppression of...
7.
Kopito R, Ron D
Nat Cell Biol . 2000 Nov; 2(11):E207-9. PMID: 11056553
A large and diverse number of diseases are now recognized as 'conformational diseases', caused by adoption of non-native protein conformations that lead to aggregation. The recent conference, 'Alpha1-antitrypsin deficiency and...
8.
Johnston J, Dalton M, Gurney M, Kopito R
Proc Natl Acad Sci U S A . 2000 Oct; 97(23):12571-6. PMID: 11050163
Deposition of aggregated protein into neurofilament-rich cytoplasmic inclusion bodies is a common cytopathological feature of neurodegenerative disease. How-or indeed whether-protein aggregation and inclusion body formation cause neurotoxicity are presently unknown....
9.
Yu H, Kopito R
J Biol Chem . 1999 Dec; 274(52):36852-8. PMID: 10601236
Unassembled alpha subunits of the T cell receptor (TCRalpha) are degraded by proteasomes following their dislocation from the endoplasmic reticulum membrane. We previously demonstrated that a variant of TCRalpha lacking...
10.
Harrington M, Gunderson K, Kopito R
J Biol Chem . 1999 Sep; 274(39):27536-44. PMID: 10488089
Gating of the cystic fibrosis Cl(-) channel requires hydrolysis of ATP by its nucleotide binding folds, but how this process controls the kinetics of channel gating is poorly understood. In...