James Shorter Department of Biochemistry and Biophysics Perelman School of Medicine at the University of Pennsylvania Biography Publications Institution JoVE Articles James Shorter has not added a biography. If you are James Shorter and would like to personalize this page please email our Author Liaison for assistance. Publications Counteracting Semen-mediated Enhancement of HIV Infection and Enveloped Virus Infection by a Lysine-specific Molecular Tweezer AIDS Research and Human Retroviruses. Oct, 2014 | Pubmed ID: 25357741 Suramin Inhibits Hsp104 ATPase and Disaggregase Activity PloS One. 2014 | Pubmed ID: 25299406 Specific Aromatic Foldamers Potently Inhibit Spontaneous and Seeded Aβ42 and Aβ43 Fibril Assembly The Biochemical Journal. Nov, 2014 | Pubmed ID: 25142005 Potentiated Hsp104 Variants Suppress Toxicity of Diverse Neurodegenerative Disease-linked Proteins Disease Models & Mechanisms. Oct, 2014 | Pubmed ID: 25062688 A Cellular System That Degrades Misfolded Proteins and Protects Against Neurodegeneration Molecular Cell. Jul, 2014 | Pubmed ID: 24882209 Reversing Deleterious Protein Aggregation with Re-engineered Protein Disaggregases Cell Cycle (Georgetown, Tex.). May, 2014 | Pubmed ID: 24694655 ALS-associated Mutation FUS-R521C Causes DNA Damage and RNA Splicing Defects The Journal of Clinical Investigation. Mar, 2014 | Pubmed ID: 24509083 Potentiated Hsp104 Variants Antagonize Diverse Proteotoxic Misfolding Events Cell. Jan, 2014 | Pubmed ID: 24439375 Conserved Distal Loop Residues in the Hsp104 and ClpB Middle Domain Contact Nucleotide-binding Domain 2 and Enable Hsp70-dependent Protein Disaggregation The Journal of Biological Chemistry. Jan, 2014 | Pubmed ID: 24280225 Disease Mutations in the Prion-like Domains of HnRNPA1 and HnRNPA2/B1 Introduce Potent Steric Zippers That Drive Excess RNP Granule Assembly Rare Diseases (Austin, Tex.). 2013 | Pubmed ID: 25002999 The Metazoan Protein Disaggregase and Amyloid Depolymerase System: Hsp110, Hsp70, Hsp40, and Small Heat Shock Proteins Prion. Nov-Dec, 2013 | Pubmed ID: 24401655 Hsp104 Suppresses Polyglutamine-induced Degeneration Post Onset in a Drosophila MJD/SCA3 Model PLoS Genetics. 2013 | Pubmed ID: 24039611 Fission Yeast Does Not Age Under Favorable Conditions, but Does So After Stress Current Biology : CB. Oct, 2013 | Pubmed ID: 24035542 Stress Granules As Crucibles of ALS Pathogenesis The Journal of Cell Biology. Apr, 2013 | Pubmed ID: 23629963 Mutations in Prion-like Domains in HnRNPA2B1 and HnRNPA1 Cause Multisystem Proteinopathy and ALS Nature. Mar, 2013 | Pubmed ID: 23455423 The Surprising Role of Amyloid Fibrils in HIV Infection Biology. 2012 | Pubmed ID: 24832047 Hsp104 Drives "protein-only" Positive Selection of Sup35 Prion Strains Encoding Strong [PSI(+)] Chemistry & Biology. Nov, 2012 | Pubmed ID: 23177195 Operational Plasticity Enables Hsp104 to Disaggregate Diverse Amyloid and Nonamyloid Clients Cell. Nov, 2012 | Pubmed ID: 23141537 Inhibition of RNA Lariat Debranching Enzyme Suppresses TDP-43 Toxicity in ALS Disease Models Nature Genetics. Dec, 2012 | Pubmed ID: 23104007 RNA-Binding Proteins in Amyotrophic Lateral Sclerosis and Neurodegeneration Neurology Research International. 2012 | Pubmed ID: 22919483 Small Heat Shock Proteins Potentiate Amyloid Dissolution by Protein Disaggregases from Yeast and Humans PLoS Biology. 2012 | Pubmed ID: 22723742 Evaluating the Role of the FUS/TLS-related Gene EWSR1 in Amyotrophic Lateral Sclerosis Human Molecular Genetics. Apr, 2012 | Pubmed ID: 22454397 The Tip of the Iceberg: RNA-binding Proteins with Prion-like Domains in Neurodegenerative Disease Brain Research. Jun, 2012 | Pubmed ID: 22445064 Hsp104 和 ClpB 可望而不可及的中间域: 位置和功能。 Biochimica Et Biophysica Acta. Jan, 2012 | Pubmed ID: 21843558 酵母功能屏幕预测新候选 ALS 疾病基因。 Proceedings of the National Academy of Sciences of the United States of America. Dec, 2011 | Pubmed ID: 22065782 哺乳动物 Disaggregase 机械: Hsp110 淡化与热休克蛋白 70 和 Hsp40 在无细胞系统中催化蛋白质分类和重新激活。 PloS One. 2011 | Pubmed ID: 22022600 RNA 结合蛋白与 ALS 和 FTLD 美国朊病毒样域 Prion. Jul, 2011 | Pubmed ID: 21847013 震慑: 释放热休克反应,治疗亨廷顿病。 The Journal of Clinical Investigation. Aug, 2011 | Pubmed ID: 21785212 分子决定因素和遗传修饰符的聚合和 ALS 病蛋白 FUS/TLS 的毒性。 PLoS Biology. Apr, 2011 | Pubmed ID: 21541367 打击与最小的药物鸡尾酒的淀粉样多态性及耐药性。 Prion. Oct-Dec, 2010 | Pubmed ID: 20935457 出现和朊病毒耐药的自然选择。 Molecular BioSystems. Jul, 2010 | Pubmed ID: 20422111 朊病毒样障碍: 模糊传递特性和传染性之间的鸿沟。 Journal of Cell Science. Apr, 2010 | Pubmed ID: 20356930 将 Hsp104 应用于蛋白质折叠异常的疾病。 Biochemistry and Cell Biology = Biochimie Et Biologie Cellulaire. Feb, 2010 | Pubmed ID: 20130674 协同的小分子结合直接根除各种朊病毒株结构。 Nature Chemical Biology. Dec, 2009 | Pubmed ID: 19915541 N 终端域征求功能 Pmel17 淀粉样纤维的形成。 The Journal of Biological Chemistry. Dec, 2009 | Pubmed ID: 19840945 TDP 43 是本质上容易聚合,和肌萎缩侧索硬化与突变加速聚合和增加毒性。 The Journal of Biological Chemistry. Jul, 2009 | Pubmed ID: 19465477 马达蛋白线程通过 Hsp104 的机制。 Molecular Cell. Apr, 2009 | Pubmed ID: 19362537 朊病毒 Proteostasis: Hsp104 满足其配角。 Prion. Oct-Dec, 2008 | Pubmed ID: 19242125 Mad2 部分展开模型: 调节细胞有丝分裂通过 Mad2 构象切换。 The Journal of Cell Biology. Dec, 2008 | Pubmed ID: 19029339 Hsp104、 热休克蛋白 70 和 Hsp40 相互作用规管形成、 增长和消除 Sup35 朊病毒。 The EMBO Journal. Oct, 2008 | Pubmed ID: 18833196 PDZ 绑定母题控制基底外侧针对龙萃-1 沿极化的上皮细胞中的生物合成途径。 Traffic (Copenhagen, Denmark). Nov, 2008 | Pubmed ID: 18764819 Hsp104 拮抗 α-synuclein 聚合并减少帕金森病大鼠模型中的多巴胺能变性。 The Journal of Clinical Investigation. Sep, 2008 | Pubmed ID: 18704197 逸出淀粉样的命运。 Nature Structural & Molecular Biology. Jun, 2008 | Pubmed ID: 18523464 直接和选择性消除特定朊病毒和淀粉类的 4,5-dianilinophthalimide 和类似物。 Proceedings of the National Academy of Sciences of the United States of America. May, 2008 | Pubmed ID: 18480256 Hsp110 伴侣调节朊病毒形成和传播在酿酒酵母中的由两个互不关联的活动。 PloS One. 2008 | Pubmed ID: 18335038 帕金森病的蛋白质 α-synuclein 扰乱了蜂窝 Rab 动态平衡。 Proceedings of the National Academy of Sciences of the United States of America. Jan, 2008 | Pubmed ID: 18162536 Hsp104: 一种武器,打击各种神经退行性疾病。 Neuro-Signals. 2008 | Pubmed ID: 18097161 非典型 AAA + 亚包装的蛋白重塑因子 Hsp104 创建扩大的腔的分类。 Cell. Dec, 2007 | Pubmed ID: 18160044 Α-synuclein 的黄金时段。 The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. Mar, 2007 | Pubmed ID: 17344380 非对称减速的 ClpB 或 Hsp104 ATPase 活性希求蛋白重塑的活动。 Nature Structural & Molecular Biology. Feb, 2007 | Pubmed ID: 17259993 销毁或催化类似 Hsp104 重塑活动的不同朊病毒的长时程增强。 Molecular Cell. Aug, 2006 | Pubmed ID: 16885031 朊病毒作为自适应管道的内存和继承。 Nature Reviews. Genetics. Jun, 2005 | Pubmed ID: 15931169 神秘 Rab1 绑定站点中 P115 中继蛋白。 The Journal of Biological Chemistry. Jul, 2005 | Pubmed ID: 15878873 导航到解决方案的 ClpB 通道。 Nature Structural & Molecular Biology. Jan, 2005 | Pubmed ID: 15689967 Hsp104 催化形成和自我复制 Sup35 消除朊病毒人云亦云。 Science (New York, N.Y.). Jun, 2004 | Pubmed ID: 15155912 GRASP65 Mitotically 受规管的高尔基堆叠因子作为直接作用。 The EMBO Journal. Jul, 2003 | Pubmed ID: 12839990 高尔基的体系结构和继承。 Annual Review of Cell and Developmental Biology. 2002 | Pubmed ID: 12142281 序贯网罗拆装门-16-GOS-28 复杂介导不同 NSF 活动驱动器高尔基膜融合。 The Journal of Cell Biology. Jun, 2002 | Pubmed ID: 12070132 序贯恃 Golgins 和 SNAREpin 大会的催化作用的囊泡中继的蛋白 P115。 The Journal of Cell Biology. Apr, 2002 | Pubmed ID: 11927603 隔离增效变种的Hsp104利用酵母Proteinopathy模型 Meredith E. Jackrel1, Amber Tariq1, Keolamau Yee1, Rachel Weitzman1, James Shorter1 1Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania Biology Hsp104纯化,蛋白质Disaggregase Elizabeth A. Sweeny1, Morgan E. DeSantis1, James Shorter1 1Department of Biochemistry and Biophysics, University of Pennsylvania JoVE 3190 Biology
隔离增效变种的Hsp104利用酵母Proteinopathy模型 Meredith E. Jackrel1, Amber Tariq1, Keolamau Yee1, Rachel Weitzman1, James Shorter1 1Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania Biology
Hsp104纯化,蛋白质Disaggregase Elizabeth A. Sweeny1, Morgan E. DeSantis1, James Shorter1 1Department of Biochemistry and Biophysics, University of Pennsylvania JoVE 3190 Biology