PubRank

James Shorter

Author PubWeight™ 67.07‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS. Nature 2013 4.03
2 The Parkinson's disease protein alpha-synuclein disrupts cellular Rab homeostasis. Proc Natl Acad Sci U S A 2007 3.39
3 TDP-43 is intrinsically aggregation-prone, and amyotrophic lateral sclerosis-linked mutations accelerate aggregation and increase toxicity. J Biol Chem 2009 3.29
4 The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease. Brain Res 2012 3.00
5 Golgi architecture and inheritance. Annu Rev Cell Dev Biol 2002 2.87
6 Stress granules as crucibles of ALS pathogenesis. J Cell Biol 2013 2.80
7 Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLS. PLoS Biol 2011 2.70
8 A yeast functional screen predicts new candidate ALS disease genes. Proc Natl Acad Sci U S A 2011 2.68
9 Prion-like disorders: blurring the divide between transmissibility and infectivity. J Cell Sci 2010 2.29
10 A direct role for GRASP65 as a mitotically regulated Golgi stacking factor. EMBO J 2003 2.15
11 Operational plasticity enables hsp104 to disaggregate diverse amyloid and nonamyloid clients. Cell 2012 2.03
12 Asymmetric deceleration of ClpB or Hsp104 ATPase activity unleashes protein-remodeling activity. Nat Struct Mol Biol 2007 1.77
13 Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosis. Hum Mol Genet 2012 1.62
14 Atypical AAA+ subunit packing creates an expanded cavity for disaggregation by the protein-remodeling factor Hsp104. Cell 2007 1.56
15 RNA-binding proteins with prion-like domains in ALS and FTLD-U. Prion 2011 1.40
16 Hsp104 antagonizes alpha-synuclein aggregation and reduces dopaminergic degeneration in a rat model of Parkinson disease. J Clin Invest 2008 1.38
17 A cryptic Rab1-binding site in the p115 tethering protein. J Biol Chem 2005 1.35
18 Motor mechanism for protein threading through Hsp104. Mol Cell 2009 1.33
19 Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease models. Nat Genet 2012 1.31
20 Small heat shock proteins potentiate amyloid dissolution by protein disaggregases from yeast and humans. PLoS Biol 2012 1.29
21 N-terminal domains elicit formation of functional Pmel17 amyloid fibrils. J Biol Chem 2009 1.28
22 A synergistic small-molecule combination directly eradicates diverse prion strain structures. Nat Chem Biol 2009 1.27
23 Hsp110 chaperones regulate prion formation and propagation in S. cerevisiae by two discrete activities. PLoS One 2008 1.26
24 ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects. J Clin Invest 2014 1.23
25 Sequential SNARE disassembly and GATE-16-GOS-28 complex assembly mediated by distinct NSF activities drives Golgi membrane fusion. J Cell Biol 2002 1.20
26 Applying Hsp104 to protein-misfolding disorders. Biochem Cell Biol 2010 1.18
27 Fission yeast does not age under favorable conditions, but does so after stress. Curr Biol 2013 1.12
28 The elusive middle domain of Hsp104 and ClpB: location and function. Biochim Biophys Acta 2011 1.09
29 Prime time for alpha-synuclein. J Neurosci 2007 1.07
30 Direct and selective elimination of specific prions and amyloids by 4,5-dianilinophthalimide and analogs. Proc Natl Acad Sci U S A 2008 1.06
31 Prion proteostasis: Hsp104 meets its supporting cast. Prion 2008 1.02
32 Escaping amyloid fate. Nat Struct Mol Biol 2008 1.01
33 The Mad2 partial unfolding model: regulating mitosis through Mad2 conformational switching. J Cell Biol 2008 0.97
34 Hsp104 suppresses polyglutamine-induced degeneration post onset in a drosophila MJD/SCA3 model. PLoS Genet 2013 0.97
35 A PDZ-binding motif controls basolateral targeting of syndecan-1 along the biosynthetic pathway in polarized epithelial cells. Traffic 2008 0.96
36 Engineering enhanced protein disaggregases for neurodegenerative disease. Prion 2015 0.96
37 Hsp104 drives "protein-only" positive selection of Sup35 prion strains encoding strong [PSI(+)]. Chem Biol 2012 0.95
38 Conserved distal loop residues in the Hsp104 and ClpB middle domain contact nucleotide-binding domain 2 and enable Hsp70-dependent protein disaggregation. J Biol Chem 2013 0.94
39 Navigating the ClpB channel to solution. Nat Struct Mol Biol 2005 0.86
40 Countering amyloid polymorphism and drug resistance with minimal drug cocktails. Prion 2010 0.83
41 Purification of hsp104, a protein disaggregase. J Vis Exp 2011 0.81
42 RNA-Binding Proteins in Amyotrophic Lateral Sclerosis and Neurodegeneration. Neurol Res Int 2012 0.81
43 Specific aromatic foldamers potently inhibit spontaneous and seeded Aβ42 and Aβ43 fibril assembly. Biochem J 2014 0.79
44 Shock and awe: unleashing the heat shock response to treat Huntington disease. J Clin Invest 2011 0.76