PUBLICATIONS

L. Guo*, J.R. Mann, J.C. Mauna, K.E. Copley, H. Wang, J.D. Rubien, H.M. Odeh, J. Lin, B.L. Lee, L. Ganser, E. Robinson, K.M. Kim, A.C. Murthy, T. Paul, B. Portz, A.M. Gleixner, Z. Diaz, J.L. Carey, A. Smirnov, G. Padilla, E. Lavorando, C. Espy, Y. Shang, E.J. Huang, A. Chesi, N.L. Fawzi, S. Myong, C.J. Donnelly*, and J. Shorter*. Defining RNA oligonucleotides that reverse deleterious phase transitions of RNA-binding proteins with prion-like domains. bioRxiv. doi:10.1101/2023.09.04.555754. pdf file link (*Co-corresponding author).

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M. E. Cicardi, V. Kankate, S. Sriramoji, K. Krishnamurthy, S. S. Markandaiah, B. M. Verdone, A. Girdhar, A. Nelson, L. B. Rivas, A. Boehringer, A. R. Haeusler, P. Pasinelli, L. Guo*, and D. Trotti*. The nuclear import receptor Kapβ2 modifies neurotoxicity mediated by poly(GR) in C9orf72-linked ALS/FTD.  bioRxiv: 2022.2009.2030.510384. pdf file link (*Co-corresponding authors).

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K. Sharma, S. Banerjee, D. Savran, C. Rajes, S. Wiese, A. Girdhar, N. Schwierz, C. Lee, J. Shorter, M. Schmidt, L. Guo, and M. Fändrich. Cryo-EM structure of the full-length hnRNPA1 amyloid fibril. J. Mol. Biol. 2023, 435(18):168211. pdf file link

J. Gao*, Q. T. Mewborne*, A. Girdhar, U. Sheth, A. N. Coyne Ritika Punathil, B. G. Kang, M. Dasovich, A. Veire, M. D. Hernandez, S Liu, Z Shi, R. Dafinca, E. Fouquerel, K. Talbot, T-I Kam, Y-J Zhang, D. Dickson, L. Petrucelli, M. van Blitterswijk, L. Guo, T. M. Dawson, V. L. Dawson, A. K. L. Leung, T. E. Lloyd, T. F. Gendron, J. D. Rothstein, K. Zhang. Poly(ADP-ribose) promotes toxicity of C9ORF72 arginine-rich dipeptide repeat proteins. Sci. Transl. Med. 2022, 14, eabq3215. pdf file link (*Co-first author).

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A. Girdhar and L. Guo. Regulating Phase Transition in Neurodegenerative Diseases by Nuclear Import Receptors. Biology, 2022, 11, 1009. pdf file link

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H.J. Kim*, P. Mohassel*, S. Donkervoort, L. Guo, K. O’Donovan, M. Coughlin, X. Lornage, N. Foulds, S.R. Hammans, A.R. Foley, C.M. Fare, A.F. Ford, M. Ogasawara, A. Sato, A. Iida, P. Munot, G. Ambegaonkar, R. Phadke, D.G. O’Donovan, R. Buchert, M. Grimmel, A. Töpf, I.T. Zaharieva, L. Brady, Y. Hu, T.E. Lloyd, A. Klein, M. Steinlin, A. Kuster, S. Mercier, P. Marcorelles, Y. Péréon, E. Fleurence, A. Manzur, S. Ennis, R. Upstill-Goddard, L. Bello, C. Bertolin, E. Pegoraro, L. Salviati, C.E. French, A. Shatillo, F.L. Raymond, T. Haack, S. Quijano-Roy, J. Böhm, I. Nelson, T. Stojkovic, T. Evangelista, V. Straub, N.B. Romero, J. Laporte, F. Muntoni, I. Nishino, M.A. Tarnopolsky, J. Shorter, J.P. Taylor, and C.G. Bönnemann. Specific heterozygous frameshift variants in hnRNPA2B1 cause early-onset oculopharyngeal muscular dystrophy. Nature Comm. 2022, 13(1):2306. pdf file link (*Co-first author).

J. L. Carey, L. Guo. Liquid-Liquid Phase Separation of TDP-43 and FUS in Physiology and Pathology of Neurodegenerative Diseases. Front. Mol. Biosci. 2022, 9:826719. doi: 10.3389/fmolb.2022.826719. pdf file link

L. Gottlieb, L. Guo, J. Shorter, and R. Marmorstein. N-alpha-acetylation of Huntingtin protein increases its propensity to aggregate. J. Biol. Chem. 2021, 297(6):101363. pdf file link

D. Beijer, *, H. J. Kim*, L. Guo*, K. O’Donovan, I. Mademan, T. Deconinck, K Van Schil, C. M. Fare, L. E. Drake, A. F. Ford, A. Kochański, D. Kabzińska, N. Dubuisson, P. Van den Bergh, N. C. Voermans, R. J. L. F. Lemmers, S. M. van der Maarel, D. Bonner, J. B. Sampson, M. T. Wheeler, A. Mehrabyan, S. Palmer, P. De Jonghe, J. Shorter, J. P. Taylor, and J. Baets. Characterization of HNRNPA1 mutations defines diversity in pathogenic mechanisms and clinical presentation. JCI Insight. 2021, 6(14):e148363. pdf file link (*Co-first author)

T. Yoshizawa, L. Guo. Karyopherin-βs play a key role as a phase separation regulator. J. Biochem. 2021, 170(1):15-23. pdf file link

E. Robinson, J. Shorter, L. Guo. Karyopherin-β2 Inhibits and Reverses Aggregation and Liquid-liquid Phase Separation of the ALS/FTD-Associated Protein FUS. Bio-protocol. 2020, 10 (16):e3725-e3725. pdf file link

C. Wang*, Y. Duan*, G. Duan*, Q. Wang, K. Zhang, X. Deng, B. Qian, J. Gu, Z. Ma, S. Zhang, L. Guo, C. Liu, Y. Fang. Stress Induces Dynamic, Cytotoxicity-Antagonizing TDP-43 Nuclear Bodies via Paraspeckle LncRNA NEAT1-Mediated Liquid-Liquid Phase Separation. Molecular Cell. 2020, 79(3):443-458. pdf file link (*Co-first author)

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A.G. Niaki*, J. Sarkar*, X. Cai, K. Rhine, V. Vidaurre, B. Guy, M. Hurst, J.C. Lee, H.R. Koh, L. Guo, C.M. Fare, J. Shorter, and S. Myong. (2020). Loss of dynamic RNA interaction and aberrant phase separation induced by two distinct types of ALS/FTD-linked FUS mutations. Mol. Cell. 77(1):82-94. pdf file link (*Co-first author).

F. Gasset-Rosa, S. Lu, H. Yu, C. Chen, Z. Melamed, L. Guo, J. Shorter, S. Da Cruz, and D.W. Cleveland. Cytoplasmic TDP-43 de-mixing independent of stress granules drives inhibition of nuclear import, loss of nuclear TDP-43, and cell death. Neuron. 2019,doi:10.1016/j.neuron.2019.02.038. pdf file link

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J.R. Mann, A.M. Gleixner, J.C. Mauna, E. Gomes, M.R. DeChellis-Marks, P.G. Needham, K.E. Copley, B. Hurtle, B. Portz, N.J. Pyles, L. Guo, C.B. Calder, Z.P. Wills, U.B. Pandey, J.K. Kofler, J.L. Brodsky, A. Thathiah, J. Shorter, and C.J. Donnelly. RNA binding antagonizes neurotoxic phase transitions of TDP-43. Neuron. 2019,doi:10.1016/j.neuron.2019.01.048. pdf file link

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Y-J. Zhang, L. Guo, P.K. Gonzales, T.F. Gendron, Y. Wu, K. Jansen-West, A.D. O’Raw, S.R. Pickles, M. Prudencio, Y. Carlomagno, M.A. Gachechiladze, C. Ludwig, R. Tian, J. Chew, M. DeTure, W-L. Lin, J. Tong, L.M. Daughrity, M. Yue, Y. Song, J.W. Andersen, M. Castanedes-Casey, A. Kurti, A. Datta, G. Antognetti, A. McCampbell, R. Rademakers, B. Oskarsson, D.W. Dickson, M. Kampmann, M.E. Ward, J.D. Fryer, C.D. Link, J. Shorter, and L. Petrucelli. Heterochromatin anomalies and double-stranded RNA accumulation underlie C9orf72 poly(PR) toxicity. Science. 2019,363(6428):eaav2606. pdf file link

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L. Guo*, C.M. Fare*, and J. Shorter. Therapeutic dissolution of aberrant phases by nuclear-import receptors. Trends. Cell Biol. 2019,doi:10.1016/j.tcb.2018.12.004. pdf file link (*Co-first author).

L. McGurk*, E. Gomes*, L. Guo*, J. Shorter, and N.M. Bonini. Poly(ADP-ribose) engages the TDP-43 nuclear-localization sequence to regulate granulo-filamentous aggregation. Biochemistry. 2018, 57(51):6923−6926. pdf file link (*Co-first author.).

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L. McGurk, E. Gomes, L. Guo, J. Mojsilovic-Petrovic, V. Tran, R.G. Kalb, J. Shorter, and N.M. Bonini. Poly(ADP-ribose) prevents aberrant phase separation of TDP-43 by promoting liquid demixing and stress-granule localization. Mol Cell. 2018, 71(5):703-717. pdf file link

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E. Bogaert, S. Boeynaems, M. Kato, L. Guo, T.R. Caulfield, J. Steyaert, W. Scheveneels, N. Wilmans, W. Haeck, N. Hersmus, J. Schymkowitz, F. Rousseau, J. Shorter, P. Callaerts, W. Robberecht, P. Van Damme, and L. Van Den Bosch. Molecular dissection of FUS points at synergistic effect of low-complexity domains in toxicity. Cell Rep. 2018, 24(3):529-537 pdf file link cover

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L. Guo*, H.J. Kim*, H. Wang*, J. Monaghan°, F. Freyermuth°, J.C. Sung°, K. O’Donovan, C.M. Fare, Z. Diaz, N. Singh, Z.C. Zhang, M. Coughlin, E.A. Sweeny, M.E. DeSantis, M.E. Jackrel, C.B. Rodell, J.A. Burdick, O.D. King, A.D. Gitler, C. Lagier-Tourenne, U.B. Pandey, Y.M. Chook, J.P. Taylor, and J. Shorter. Nuclear-import receptors reverse aberrant phase transitions of RNA-binding proteins with prion-like domains. Cell. 2018, 173(3):677-692. pdf file link (*Co-first author. °Co-second author).

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T. Zhang*, Y-C. Wu*, P. Mullane, Y.J. Ji, H. Liu, L. He, A. Arora, H-Y. Hwang, A.F. Alessi, A.G. Niaki, G. Periz, L. Guo, H. Wang, E. Elkayam, L. Joshua-Tor, S. Myong, J.K. Kim, J. Shorter, S-E. Ong, A.K.L. Leung, and J. Wang. FUS regulates activity of microRNA-mediated gene silencing. Mol. Cell. 2018, 69(5):787-801. pdf file link(*Co-first author).

L. Guo and J. Shorter. Biology and pathobiology of TDP-43 and emergent therapeutic strategies. Cold Spring Harb Perspect Med. 2017, 7(9). pii: a024554. pdf file link

L. Guo and J. Shorter. It's raining liquids: RNA tunes viscoelasticity and dynamics of membraneless organelles. Mol. Cell. 2015, 60, 189-192. pdf file link

L. Guo and F. Gai. Simple Method to Enhance the Photostability of the Fluorescence Reporter R6G for Prolonged Single-Molecule Studies. J. Phys. Chem. A. 2013, 117, 6164-6170. pdf link

D. Chow, L. Guo, F. Gai, and M. Goulian. Fluorescence correlation spectroscopy measurements of the membrane protein TetA in Escherichia coli suggest rapid diffusion at short length scales. PLoS ONE. 2012, 7(10):e48600. pdf link

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Y-H. Wang, A. Collins, L. Guo, K. B. Smith-Dupont, F. Gai, T. Svitkina and P. A. Janmey. Divalent cation-induced cluster formation by polyphosphoinositides in model membranes. J. Am. Chem. Soc. 2012, 134, 3387-3395. pdf link

L. Guo, K. B. Smith-Dupont and F. Gai. Diffusion as a probe of peptide-induced membrane domain formation. Biochemistry. 2011, 50, 2291-2297. pdf link

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J. M. G. Rogers*, A. Polishchuk*, L. Guo*, J. Wang, W. F. DeGrado and F. Gai. Photoinduced electron transfer and fluorophore motion as a probe of the conformational dynamics of membrane proteins: application to the influenza A M2 proton channel. Langmuir. 2011, 27, 3815-3821. (*Equal Contribution). pdf link