2014

67. Jackrel, M.E., A. Tariq, K. Yee, R. Weitzman, and J. Shorter^. (2014). Isolating potentiated Hsp104 variants using yeast proteinopathy models. J. Vis. Exp. In press. (^Corresponding author).

66. Jackrel, M.E., and J. Shorter^. (2014). Potentiated Hsp104 variants suppress toxicity of diverse neurodegenerative disease-linked proteins. Dis. Model Mech. doi: 10.1242/dmm.016113. pdf file link (^Corresponding author).

65. Guo, L. B.I. Giasson, A. Glavis-Bloom, M.D. Brewer, J. Shorter, A.D. Gitler, and X. Yang. (2014). A cellular system that degrades misfolded proteins through sequential sumoylation and ubiquitination. Mol. Cell. 55(1):15-30. pdf file link cover

64. Jackrel, M.E., and J. Shorter^. (2014). Reversing deleterious protein aggregation with re-engineered protein disaggregases. Cell Cycle. 13(9):1379-1383. pdf file link (^Corresponding author).

63. Qiu, H., S. Lee, Y. Shang, W.Y. Wang, K.F. Au, S. Kamiya, S. J. Barmada, S. Finkbeiner, H. Lui, C.E. Carlton, A.A. Tang, M.C. Oldham, H. Wang, J. Shorter, A.J. Filiano, E.D. Roberson, W.G. Tourtellotte, B. Chen, L.H. Tsai, and E.J. Huang. (2014). ALS Mutation FUS-R521C Causes DNA Damage and RNA Splicing Defects. J. Clin. Invest. 124(3):981–999. pdf file link

62. Jackrel, M.E., M.E. DeSantis, B.A. Martinez, L.M. Castellano, R.M. Stewart, K.A. Caldwell, G.A. Caldwell, and J. Shorter^. (2014). Potentiated Hsp104 variants antagonize diverse proteotoxic misfolding events. Cell. 156(1-2):170–182. pdf file link (^Corresponding author).

61. DeSantis, M.E., E.A. Sweeny, D. Snead, E.H. Leung, M.S. Go, K. Gupta, P. Wendler, and J. Shorter^. (2014). 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. 289(2):848-867. pdf file link (^Corresponding author).

2013

60. Torrente, M.P., and J. Shorter^. (2013). The metazoan protein disaggregase and amyloid depolymerase system: Hsp110, Hsp70, Hsp40, and small heat shock proteins. Prion. 7(6):457-463. pdf file link (^Corresponding author).

59. Coelho, M., A. Dereli, A. Haese, S. Kühn, L. Malinovska, M.E. DeSantis, J. Shorter, S. Alberti, T. Gross, and I.M. Tolić-Nørrelykke. (2013). Fission yeast does not age under favorable conditions, but does so after stress. Curr. Biol. 23(19): 1844-1852. pdf file link

58. Cushman-Nick, M., N.M. Bonini^, and J. Shorter^. (2013). Hsp104 suppresses polyglutamine-induced degeneration post onset in a Drosophila MJD/SCA3 model. PLoS Genetics. 9(9): e1003781. (^Co-corresponding author). pdf file link

57. Shorter, J.^, and J.P. Taylor^. (2013). Disease mutations in the prion-like domains of hnRNPA1 and hnRNPA2B1 introduce potent steric zippers that drive excess RNP granule assembly. Rare Diseases. 1(2):e25200. pdf file link (^Co-corresponding author).

56. Li, Y.R.^, O.D. King, J. Shorter^, and A.D. Gitler^. (2013). Stress granules as crucibles of ALS pathogenesis. J. Cell Biol. 201(3):361-372. pdf file link (^Co-corresponding author).

55. Kim*, H.J., N.C.Kim*, Y.D. Wang*, E.A. Scarborough*, J. Moore*, Z. Diaz*, K.S. MacLea, B. Freibaum, S. Li, A. Molliex, A.P. Kanagaraj, R. Carter, K.B. Boylan, A.M. Wojtas, R. Rademakers, J.L. Pinkus, S.A. Greenberg, J.Q. Trojanowski, B.J. Traynor, B.N. Smith, S. Topp, A.S. Gkazi, J. Miller, C.E. Shaw, M. Kottlors, J. Kirschner, A. Pestronk, Y.R. Li, A.F. Ford, A.D. Gitler, M. Benatar, O.D. King, V.E. Kimonis, E.D. Ross, C.C. Weihl, J. Shorter^, and J.P. Taylor^. (2013). Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS. Nature. 495(7442):467–473. pdf file link (*Co-first author. ^Co-corresponding author).

2012

54. DeSantis, M.E., and J. Shorter^. (2012). Hsp104 Drives ''Protein-Only'' Positive Selection of Sup35 Prion Strains Encoding Strong [PSI+]. Chemistry & Biology. 19(11):1400-1410. pdf file link (^Corresponding author).

53. DeSantis, M.E., E.H. Leung, E.A. Sweeny, M.E. Jackrel, M. Cushman-Nick, A. Neuhaus-Follini, S. Vashist, M.A. Sochor, M.N. Knight, and J. Shorter^. (2012). Operational Plasticity Enables Hsp104 to Disaggregate Diverse Amyloid and Nonamyloid Clients. Cell. 151(4):778-793. pdf file link (^Corresponding author).

52. Armakola, M., Higgins, M.J., Figley, M.D., Barmada, S.J., Scarborough, E.A., Diaz, Z., Fang, X., Shorter, J., Krogan, N.J., Finkbeiner, S., Farese Jr., R.V., and Gitler, A.D. (2012). Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease models. Nat. Genet. 44(12):1302–1309. pdf file link

51. Duennwald, M.L., A.L. Echeverria, and J. Shorter^. (2012). Small heat shock proteins potentiate amyloid dissolution by protein disaggregases from yeast and humans. PLoS Biol. 9(6):e1001346. pdf file link (^Corresponding author).

50. Castellano, L.M., and J. Shorter^. (2012). The surprising role of amyloid fibrils in HIV infection. Biology. 1(1):58-80. pdf file link (^Corresponding author).

49. Couthouis, J., M.P. Hart, R. Erion, Z. Diaz, T. Nakaya, F. Ibrahim, H.J. Kim, J. Mojsilovic-Petrovic, D. Clay-Falcone, L. Elman, L. McCluskey, R. Greene, R.G. Kalb, V.M. Lee, J.Q. Trojanowski, G.A. Nicholson, I.P. Blair, O.D. King, N.M. Bonini, V.M. Van Deerlin, Z. Mourelatos, J. Shorter, and A.D. Gitler. (2012). Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosis. Hum. Mol. Genet. 21(13):2899-911. pdf file link

48. Ugras, S.E., and J. Shorter^. (2012). RNA-binding proteins in amyotrophic lateral sclerosis and neurodegeneration. Neurol. Res. Int. doi:10.1155/2012/432780. pdf file link (^Corresponding author).

47. King, O.D.^, A.D. Gitler^, and J. Shorter^. (2012). The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease. Brain Res. 1462:61-80. pdf file link (^Co-corresponding authors).

46. DeSantis, M.E., and J. Shorter^. (2012). The elusive middle domain of Hsp104 and ClpB: location and function. Biochimica et Biophysica Acta. 1823(1):29-39. pdf file link (^Corresponding author).

2011

45. Couthouis, J.*, M.P. Hart*, J. Shorter*, M. DeJesus-Hernandez, R. Erion, R.E. Oristano, X.A. Liu, D. Ramos, N. Jethava, D. Hosangadi, J. Epstein, A. Chiang, Z. Diaz, T. Nakaya, F. Ibrahim, H.J. Kim, J.A. Solski, K.L. Williams, J. Mojsilovic-Petrovic, C. Ingre, K. Boylan, N. Graff-Radford, D. Dickson, D. Clay-Falcone, L. Elman, L. McCluskey, R. Greene, R.G. Kalb, V.M.Y. Lee, J.Q. Trojanowski, A.C. Ludolph, W. Robberecht, P.M. Andersen, G.A. Nicholson, I.P. Blair, O.D. King, N.M. Bonini, V. Van Deerlin, R. Rademakers, Z. Mourelatos, and A.D. Gitler. (2011). A yeast functional screen predicts new candidate ALS disease genes. Proc. Natl. Acad. Sci. U.S.A. 108(52):20881-90. pdf file link cover (*Authors contributed equally to this work).

44. Shorter, J^. (2011). The mammalian disaggregase machinery: Hsp110 synergizes with Hsp70 and Hsp40 to catalyze protein disaggregation and reactivation in a cell-free system. PLoS ONE. 6(10):e26319. pdf file link (^Corresponding author).

43. Sweeny, E.A., M.E. DeSantis, and J. Shorter^. (2011). Purification of Hsp104, a protein disaggregase. J. Vis. Exp. (55), e3190, DOI: 10.3791/3190 pdf file link (^Corresponding author).

42. Shorter, J^. (2011). Amyloid remodeling by Hsp104, in Protein Chaperones and Protection from Neurodegeneration (ed. Witt, S.N.). Chapter 7: 235-259. (John Wiley & Sons, Inc.). pdf file link (^Corresponding author).

41. Gitler, A.D.^, and J. Shorter^. (2011). RNA-binding proteins with prion-like domains in ALS and FTLD-U. Prion. 5(3):179-187. pdf file link cover (^Co-corresponding author).

40. Jackrel, M.E., and J. Shorter^. (2011). Shock and awe: unleashing the heat shock response to treat Huntington disease. J. Clin. Invest. 121(8):2972-2975. pdf file link (^Corresponding author).

39. Sun, Z., Z. Diaz, X. Fang, M.P. Hart, A. Chesi, J. Shorter^, and A.D. Gitler^. (2011). Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLS. PLoS Biol. 9(4):e1000614. pdf file link (^Co-Corresponding author).

2010

38. Duennwald, M.L.^, and J. Shorter^. (2010). Countering amyloid polymorphism and drug resistance with minimal drug cocktails. Prion. 4(4): 244-251. pdf file link (^Co-Corresponding author).

37. Shorter, J.^ (2010). Emergence and natural selection of drug-resistant prions. Mol. Biosyst. 6(7):1115-1130. pdf file link (^Corresponding author).

36. Cushman, M., B.S. Johnson, O.D. King, A.D. Gitler^, and J. Shorter^. (2010). Prion-like disorders: blurring the divide between transmissibility and infectivity. J. Cell Sci. 123(8):1191-1201. pdf file link (^Co-Corresponding author).

35. Vashist, S., M. Cushman, and J. Shorter^. (2010). Applying Hsp104 to protein-misfolding disorders. Biochem. Cell Biol. 88(1):1-13. pdf file link (^Corresponding author).

2009

34. Roberts, B.E., M.L. Duennwald^, H. Wang, C. Chung, N.P. Lopreiato, E.A. Sweeny, M.N. Knight, and J. Shorter^. (2009). A synergistic small-molecule combination directly eradicates diverse prion strain structures. Nat. Chem. Biol. 5(12):936-946. pdf file link (^Co-Corresponding author).

33. Watt, B., G. van Niel, D.M. Fowler, I. Hurbain, K.C. Luk, S.E. Stayrook, M.A. Lemmon, G. Raposo, J. Shorter, J.W. Kelly, and M.S. Marks. (2009). N-terminal domains elicit formation of functional Pmel17 amyloid fibrils. J. Biol. Chem. 284(51):35543-35555. pdf file link cover

32. Johnson, B.S., D. Snead, J.J. Lee, J. M. McCaffery, J. Shorter^, and A.D. Gitler^. (2009). TDP-43 is intrinsically aggregation-prone and Amyotrophic Lateral Sclerosis-linked mutations accelerate aggregation and increase toxicity. J. Biol. Chem. 284(30):20329-39. pdf file link (^Co-Corresponding author).

31. Wendler, P., J. Shorter, D. Snead, C. Plisson, D.K. Clare, S. Lindquist, and H.R. Saibil. (2009). Motor mechanism for protein threading through Hsp104. Mol. Cell. 34(1):81-92. pdf file link

2008

30. Sweeny, E.A., and J. Shorter^. (2008). Prion proteostasis: Hsp104 meets its supporting cast. Prion. 2(4):135-40. pdf file link (^Corresponding author)

29. Skinner, J.J., S. Wood, J. Shorter, S.W. Englander, and B.E. Black. (2008). The Mad2 partial unfolding model: regulating mitosis through Mad2 conformational switching. J. Cell Biol. 183(5):761-768. pdf file link

28. Shorter, J.^, and S. Lindquist. (2008). Hsp104, Hsp70 and Hsp40 interplay regulates formation, growth and elimination of Sup35 prions. EMBO J. 27 (20):2712–2724. pdf file link (^Corresponding author).

27. Lo Bianco, C.*, J. Shorter*^, E. Régulier, H. Lashuel, T. Iwatsubo, S. Lindquist, and P. Aebischer^. (2008). Hsp104 antagonizes alpha-synuclein aggregation and reduces dopaminergic degeneration in a rat model of Parkinson’s disease. J. Clin. Invest. 118(9):3087-3097. pdf file link (*Authors contributed equally to this work. ^Co-Corresponding author)

26. Maday, S., E. Anderson, H.C. Chang, J. Shorter, A. Satoh, J. Sfakianos, H. Foelsch, J.M. Anderson, Z. Walther, and I. Mellman. (2008). A PDZ-binding motif controls basolateral targeting of syndecan-1 along the biosynthetic pathway in polarized epithelial cells. Traffic. 9(11):1915–1924. pdf file link

25. Roberts, B.E. and J. Shorter^. (2008). Escaping Amyloid Fate. Nat. Struct. Mol. Biol. 15(6):544-546. pdf file link (^Corresponding author)

24. Wang, H., M.L. Duennwald, B.E. Roberts, L.M. Rozeboom, Y.L. Zhang, A.D. Steele, R. Krishnan, L.J. Su, D. Griffin, S. Mukhopadhyay, E.J. Hennessy, P. Weigele, B.J. Blanchard, J. King, A.A. Deniz, S.L. Buchwald, V.M. Ingram, S. Lindquist^, and J. Shorter^. (2008). Direct and selective elimination of specific prions and amyloids by 4,5-dianilinophthalimide and analogs. Proc. Natl. Acad. Sci. U.S.A. 105(20):7159-7164. pdf file link (^Co-Corresponding author)

23. Sadlish H., H. Rampelt, J. Shorter, R.D. Wegrzyn, C. Andréasson, S. Lindquist, and B. Bukau. (2008). Hsp110 chaperones regulate prion formation and propagation in S. cerevisiae by two discrete activities. PLoS ONE. 3(3):e1763. pdf file link

22. Shorter, J.^ (2008). Hsp104: a weapon to combat diverse neurodegenerative disorders. Neurosignals. 16(1):63-74. pdf file link cover (^Corresponding author)

21. Gitler, A.D., B.J. Bevis, J. Shorter , K.E. Strathearn, S. Hamamichi, L.J. Su, K.A. Caldwell, G.A. Caldwell, J.C. Rochet, J.M. McCaffery, C. Barlowe, and S. Lindquist. (2008). The Parkinson's disease protein alpha-synuclein disrupts cellular Rab homeostasis. Proc. Natl. Acad. Sci. U.S.A. 105(1):145-150. pdf file link

2007

20. Wendler, P., J. Shorter, C. Plisson, A.G. Cashikar, S. Lindquist, and H.R. Saibil. (2007). Atypical AAA+ subunit packing creates an expanded cavity for disaggregation by the protein-remodeling factor Hsp104. Cell. 131(7):1366-1377. pdf file link

19. Gitler, A. D.^, and J. Shorter^ (2007). Prime time for alpha-synuclein. J. Neurosci. 27(10):2433-2434. pdf file link (^Co-Corresponding author)

18. Doyle, S. M.*, J. Shorter*, M. Zolkiewski, J.R. Hoskins, S. Lindquist, and S. Wickner. (2007). Asymmetric deceleration of ClpB or Hsp104 ATPase activity unleashes protein-remodeling activity. Nat. Struct. Mol. Biol. 14(2):114-122. pdf file link (*Authors contributed equally to this work)

2006

17. Shorter, J., and S. Lindquist. (2006). Destruction or potentiation of different prions catalyzed by similar Hsp104 remodeling activities. Mol. Cell. 23(3):425-438. pdf file link

2005

16. Shorter, J.^, and S. Lindquist^. (2005). Prions as adaptive conduits of memory and inheritance. Nat. Rev. Genetics. 6(6):435-450. pdf file link (^Co-Corresponding author)

15. Beard, M.B., A. Satoh, J. Shorter, and G. Warren. (2005). A cryptic Rab1-binding site in the p115 tethering protein. J. Biol. Chem. 280(27):25840-25848. pdf file link

14. Shorter, J., and S. Lindquist. (2005). Navigating the ClpB channel to solution. Nat. Struct. Mol. Biol. 12(1):4-6. pdf file link

2004

13. Shorter, J., and S. Lindquist. (2004). Hsp104 catalyzes formation and elimination of self-replicating Sup35 prion conformers. Science. 304(5678):1793-1797. pdf file link

2003

12. Wang, Y., J. Seemann, M. Pypaert, J. Shorter, and G. Warren. (2003). A direct role for GRASP65 as a mitotically regulated Golgi stacking factor. EMBO J. 22(13):3279-90. pdf file link

2002

11. Shorter, J.^, and G. Warren^. (2002). Golgi Architecture and Inheritance. Annu. Rev. Cell Dev. Biol. 18:379-420. pdf file link (^Co-Corresponding author)

10. Müller, J.M.*, J. Shorter*, R. Newman, K. Deinhardt, Y. Sagiv, Z. Elazar, G. Warren, and D.T. Shima. (2002). Sequential SNARE disassembly and GATE-16/GOS-28 complex assembly mediated by distinct NSF activities drives Golgi membrane fusion. J. Cell Biol. 157(7):1161-1173. pdf file link (*Authors contributed equally to this work)

9. Shorter, J., M.B. Beard, J. Seemann, A.B. Dirac-Svejstrup, and G. Warren. (2002). Sequential tethering of Golgins and catalysis of SNAREpin assembly by the vesicle-tethering protein p115. J. Cell Biol. 157(1):45-62. pdf file link

2000

8. Dirac-Svejstrup, A.B., J. Shorter, M.G. Waters, and G. Warren. (2000). Phosphorylation of the vesicle-tethering protein p115 by a casein kinase II-like enzyme is required for Golgi reassembly from isolated mitotic fragments. J. Cell Biol. 150(3):475-488. pdf file link

7. Meyer, H. H., J.G. Shorter, J. Seemann, D. Pappin, and G. Warren. (2000). A complex of mammalian ufd1 and npl4 links the AAA-ATPase, p97, to ubiquitin and nuclear transport pathways. EMBO J. 19(10):2181-2192. pdf file link

6. Shorter, J. (2000). Molecular mechanisms regulating Golgi architecture during the mammalian cell division cycle. Ph.D. Thesis (University of London).

5. Barr, F.A., and J. Shorter. (2000). Membrane traffic: do cones mark sites of fission? Curr. Biol. 10(4):R141-144. pdf file link

4. Lesa, G.M., J. Seemann, J. Shorter, J. Vandekerckhove, and G. Warren. (2000). The amino-terminal domain of the Golgi protein Giantin interacts directly with the vesicle-tethering protein p115. J. Biol. Chem. 275(4):2831-2836. pdf file link

1999

3. Müller, J.M., C. Rabouille, R. Newman, J. Shorter, P. Freemont, G. Schiavo, G. Warren, and D.T. Shima. (1999). An NSF function distinct from ATPase-dependent SNARE disassembly is essential for Golgi membrane fusion. Nat. Cell Biol. 1(6):335-340. pdf file link

2. Shorter, J., R. Watson, M.E. Giannakou, M. Clarke, G. Warren, and F.A. Barr. (1999). GRASP55, a second mammalian GRASP protein involved in the stacking of Golgi cisternae in a cell-free system. EMBO J. 18(18):4949-4960. pdf file link

1. Shorter, J.^, and G. Warren. (1999). A role for the vesicle tethering protein, p115, in the post-mitotic stacking of reassembling Golgi cisternae in a cell-free system. J. Cell Biol. 146(1):57-70. pdf file link (^Corresponding author)

gs pm


 



 

twitter

Department of Biochemistry & Biophysics

p3
University of Pennsylvania