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| 1 | Protein misfolding in neurodegenerative diseases:implications and strategies显示文摘A hallmark of neurodegenerative proteinopathies is the formation of misfolded protein aggregates that cause cellular toxicity and contribute to cellular proteostatic collapse.Therapeutic options are currently being explored that target different steps in the production and processing of proteins implicated in neurodegenerative disease,including synthesis,chaperone-assisted folding and trafficking,and degradation via the proteasome and autophagy pathways.Other therapies,like mTOR inhibitors and activators of the heat shock response,can rebalance the entire proteostatic network.However,there are major challenges that impact the development of novel therapies,including incomplete knowledge of druggable disease targets and their mechanism of action as well as a lack of biomarkers to monitor disease progression and therapeutic response.A notable development is the creation of collaborative ecosystems that include patients,clinicians,basic and translational researchers,foundations and regulatory agencies to promote scientific rigor and clinical data to accelerate the development of therapies that prevent,reverse or delay the progression of neurodegenerative proteinopathies. | Patrick Sweeney Hyunsun Park Marc Baumann John Dunlop Judith Frydman Ron Kopito Alexander McCampbell Gabrielle Leblanc Anjli Venkateswaran Antti Nurmi Robert Hodgson | 2017 | Translational Neurodegeneration2017，6，1： | 4 |
| 2 | The role of cysteineresidues in the erythrocyte plasma membrane anion exchange protein, AE1显示文摘 | Casey JR Ding Y Kopito RR | 1995 | J Biol Chem1995，270，15： | 2 |
| 3 | Impairment of the ubiquitin-proteasome system by protein aggregation 显示文摘 | BENCE N F SAMPAT R M KOPITO R R | 2001 | Science2001，292，5521： | 1 |
| 4 | Derlin-1 is a rhomboid pseudo-protease required for the dislocation of mutant a-1 antitrypsin from theendoplasmic reticulum显示文摘 | Greenblatt EJ Olzmann JA Kopito RR | 2011 | Nat Struct Mol Biol2011，18，10： | 1 |
| 5 | Aggresomes, inclusion bodies and protein aggregation显示文摘 | Kopito R R | 2000 | Trends in Cell Biology2000，10，： | 1 |
| 6 | Conformational disease显示文摘 | Kopito RR Ron D | | 0，，： | 1 |
| 7 | Central pore residues mediate the p97NCP activity required for ERAD显示文摘 | DeLaBarre B Christianson JC Kopito RR | 2006 | Mol Cell2006，22，4： | 1 |
| 8 | Impairment of the Ubiquitin-proteasome System by Protein Aggregation显示文摘 | SAMPAT RM KOPITO RR | 2001 | Science(S0036-8075)2001，292，5521： | 1 |
| 9 | The role of cysteine residues in the erythrocyte plasma membrane anion exchange protein,AE1显示文摘 | Casey JR Ding Y Kopito RR | 1995 | J Biol Chem1995，270，15： | 1 |
| 10 | Ocular response analyzer: feasibility study and correlation with normal eyes 显示文摘 | Montard R Kopito R Touzeau O | 2007 | J Fr Oph?talmol2007，30，： | 1 |
| 11 | Molecular biology of the anion exchanger gene family显示文摘 | Kopito RR | 1990 | Int Rev Cytol1990，123，： | 1 |
| 12 | Degradation of CFFR by the ubiquitin-proteasome pathway显示文摘 | Ward CL Omura S Kopito RR | 1995 | Cell1995，83，1： | 1 |
| 13 | Degradation of CFTR by the ubiquitin-p roteasome pathway显示文摘 | Ward CL Omura S Kopito RR | 1995 | Cell1995，83，1： | 1 |
| 14 | Impairment of the ubiquitinproteasome system by protein aggregation 显示文摘 | Bence NF Sampat RM Kopito RR | 2001 | Science2001，292，5521： | 1 |
| 15 | Impairment of the ubiquitin-proteasome system by protein aggregation显示文摘 | Bence NF Sampat RM Kopito RR | 2001 | Science2001，292，2： | 1 |
| 16 | Misfolded proteins partition between two distinet quality control compartments 显示文摘 | Kaganovieh D Kopito R Frydman J | 2008 | Nature2008，454，： | 1 |
| 17 | Biosynthesis and detradation of CFTR显示文摘 | | 1999 | Physiol Rev1999，79，： | 1 |
| 18 | Aggresomes,inclusio bodies and pro- tein aggregation显示文摘 | Kopito R R | 2000 | Trends in Cell Biology2000，10，： | 1 |
| 19 | The role of cysteine residues in the erythrocyte plasma membrane anion exchange protein, AE1显示文摘 | Casey JR Ding Y Kopito RR | 1995 | J Biol Chem1995，270，15： | 1 |
| 20 | Impairment of the ubiquitin-proteasome system by protein aggregation显示文摘 | Bence NF Sampat RM Kopito RR | 2001 | Science2001，292，5521： | 1 |
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