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Select item 114249781.

DDI2 protease controls embryonic development and inflammation via TCF11/NRF1

Monika Nedomova, Stefanie Haberecht-Müller, Sophie Möller, Simone Venz, Michaela Prochazkova, Jan Prochazka, Frantisek Sedlak, Kallayanee Chawengsaksophak, Elke Hammer, Petr Kasparek, Michael Adamek, Radislav Sedlacek, Jan Konvalinka, Elke Krüger, Klara Grantz Saskova

iScience. 2024 Oct 18; 27(10): 110893. Published online 2024 Sep 5. doi: 10.1016/j.isci.2024.110893

PMCID:: PMC11424978

Abstract Article PDF–6.1M

Select item 98231022.

Ubiquilin-2 regulates pathological alpha-synuclein

Stephanie S. Sandoval-Pistorius, Julia E. Gerson, Nyjerus Liggans, Jaimie H. Ryou, Kulin Oak, Xingli Li, Keyshla Y. Negron-Rios, Svetlana Fischer, Henry Barsh, Emily V. Crowley, Mary E. Skinner, Lisa M. Sharkey, Sami J. Barmada, Henry L. Paulson

Sci Rep. 2023; 13: 293. Published online 2023 Jan 6. doi: 10.1038/s41598-022-26899-0

PMCID:: PMC9823102

Abstract Article PDF–10M

Select item 93768613.

RTL8 promotes nuclear localization of UBQLN2 to subnuclear compartments associated with protein quality control

Harihar Milaganur Mohan, Hanna Trzeciakiewicz, Amit Pithadia, Emily V. Crowley, Regina Pacitto, Nathaniel Safren, Bryce Trotter, Chengxin Zhang, Xiaogen Zhou, Yang Zhang, Venkatesha Basrur, Henry L. Paulson, Lisa M. Sharkey

Cell Mol Life Sci. 2022 Mar; 79(3): 176. Published online 2022 Mar 5. doi: 10.1007/s00018-022-04170-z

PMCID:: PMC9376861

Abstract Article PDF–6.1M

Select item 88965464.

Disrupting the Balance of Protein Quality Control Protein UBQLN2 Accelerates Tau Proteinopathy

Julia E. Gerson, Stephanie Sandoval-Pistorius, Jacqueline P. Welday, Aleija Rodriguez, Jordan D. Gregory, Nyjerus Liggans, Kylie Schache, Xingli Li, Hanna Trzeciakiewicz, Sami Barmada, Lisa M. Sharkey, Henry L. Paulson

J Neurosci. 2022 Mar 2; 42(9): 1845–1863. doi: 10.1523/JNEUROSCI.1116-21.2021

PMCID:: PMC8896546

Abstract Article PDF–8.5M

Select item 83873695.

Serpin neuropathology in the P497S UBQLN2 mouse model of ALS/FTD

Nicole R. Higgins, Jessie E. Greenslade, Josephine J. Wu, Elena Miranda, Giovanna Galliciotti, Mervyn J. Monteiro

Brain Pathol. 2021 Sep; 31(5): e12948. Published online 2021 Mar 29. doi: 10.1111/bpa.12948

PMCID:: PMC8387369

Abstract Article PDF–4.0M

Select item 79387706.

BraInMap elucidates the macromolecular connectivity landscape of mammalian brain

Reza Pourhaghighi, Peter E. A. Ash, Sadhna Phanse, Florian Goebels, Lucas Z. M. Hu, Siwei Chen, Yingying Zhang, Shayne D. Wierbowski, Samantha Boudeau, Mohamed T. Moutaoufik, Ramy H. Malty, Edyta Malolepsza, Kalliopi Tsafou, Aparna Nathan, Graham Cromar, Hongbo Guo, Ali Al Abdullatif, Daniel J. Apicco, Lindsay A. Becker, Aaron D. Gitler, Stefan M. Pulst, Ahmed Youssef, Ryan Hekman, Pierre C. Havugimana, Carl A. White, Benjamin C. Blum, Antonia Ratti, Camron D. Bryant, John Parkinson, Kasper Lage, Mohan Babu, Haiyuan Yu, Gary D. Bader, Benjamin Wolozin, Andrew Emili

Cell Syst. Author manuscript; available in PMC 2021 Apr 22.

Published in final edited form as: Cell Syst. 2020 Apr 22; 10(4): 333–350.e14. doi: 10.1016/j.cels.2020.03.003

Correction in:: Cell Syst. 2020 Aug 26; 11(2): 208.

PMCID:: PMC7938770

Abstract Article PDF–2.0M

Select item 78737017.

Global proteomics of Ubqln2-based murine models of ALS

Alexandra M. Whiteley, Miguel A. Prado, Stefanie A.H. de Poot, Joao A. Paulo, Marissa Ashton, Sara Dominguez, Martin Weber, Hai Ngu, John Szpyt, Mark P. Jedrychowski, Amy Easton, Steven P. Gygi, Thimo Kurz, Mervyn J. Monteiro, Eric J. Brown, Daniel Finley

J Biol Chem. 2021 Jan-Jun; 296: 100153. Published online 2020 Dec 10. doi: 10.1074/jbc.RA120.015960

PMCID:: PMC7873701

Abstract Article PDF–3.0M

Select item 75049108.

Modeling UBQLN2-mediated neurodegenerative disease in mice: shared and divergent properties of wild type and mutant UBQLN2 in phase separation, subcellular localization, altered proteostasis pathways, and selective cytotoxicity

Lisa M. Sharkey, Stephanie S. Sandoval-Pistorius, Shannon J. Moore, Julia E. Gerson, Robert Komlo, Svetlana Fischer, Keyshla Y. Negron-Rios, Emily V. Crowley, Francisco Padron, Ronak Patel, Geoffrey G. Murphy, Henry L. Paulson

Neurobiol Dis. Author manuscript; available in PMC 2021 Sep 1.

Published in final edited form as: Neurobiol Dis. 2020 Sep; 143: 105016. Published online 2020 Jul 10. doi: 10.1016/j.nbd.2020.105016

PMCID:: PMC7504910

Abstract Article PDF–2.8M

Select item 74715009.

Ubiquilin-2 differentially regulates polyglutamine disease proteins

Julia E Gerson, Nathaniel Safren, Svetlana Fischer, Ronak Patel, Emily V Crowley, Jacqueline P Welday, Alexandra K Windle, Sami Barmada, Henry L Paulson, Lisa M Sharkey

Hum Mol Genet. 2020 Aug 29; 29(15): 2596–2610. Published online 2020 Jul 18. doi: 10.1093/hmg/ddaa152

PMCID:: PMC7471500

Abstract Article PDF–2.0M

Select item 733465110.

ALS/FTD mutations in UBQLN2 impede autophagy by reducing autophagosome acidification through loss of function

Josephine J. Wu, Ashley Cai, Jessie E. Greenslade, Nicole R. Higgins, Cong Fan, Nhat T. T. Le, Micaela Tatman, Alexandra M. Whiteley, Miguel A. Prado, Birger V. Dieriks, Maurice A. Curtis, Christopher E. Shaw, Teepu Siddique, Richard L. M. Faull, Emma L. Scotter, Daniel Finley, Mervyn J. Monteiro

Proc Natl Acad Sci U S A. 2020 Jun 30; 117(26): 15230–15241. Published online 2020 Jun 8. doi: 10.1073/pnas.1917371117

Correction in:: Proc Natl Acad Sci U S A. 2021 Sep 7; 118(36): e2114051118.

PMCID:: PMC7334651

Abstract Article PDF–2.0M

Select item 672539411.

Telencephalic Embryonic Subtractive Sequences: A Unique Collection of Neurodevelopmental Genes

Alessandro Bulfone, Pietro Carotenuto, Andrea Faedo, Veruska Aglio, Livia Garzia, Anna Maria Bello, Andrea Basile, Alessandra Andrè, Massimo Cocchia, Ombretta Guardiola, Andrea Ballabio, John L. R. Rubenstein, Massimo Zollo

J Neurosci. 2005 Aug 17; 25(33): 7586–7600. doi: 10.1523/JNEUROSCI.0522-05.2005

PMCID:: PMC6725394

Abstract Article PDF–682K

Select item 664747112.

Neuronal Expression of UBQLN2^P497H Exacerbates TDP-43 Pathology in TDP-43^G348C Mice through Interaction with Ubiquitin

Vincent Picher-Martel, Laurence Renaud, Christine Bareil, Jean-Pierre Julien

Mol Neurobiol. 2019; 56(7): 4680–4696. Published online 2018 Oct 30. doi: 10.1007/s12035-018-1411-3

PMCID:: PMC6647471

Abstract Article PDF–3.8M

Select item 621742113.

Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly

Lisa M. Sharkey, Nathaniel Safren, Amit S. Pithadia, Julia E. Gerson, Mark Dulchavsky, Svetlana Fischer, Ronak Patel, Gabrielle Lantis, Naila Ashraf, John H. Kim, Alia Meliki, Eiko N. Minakawa, Sami J. Barmada, Magdalena I. Ivanova, Henry L. Paulson

Proc Natl Acad Sci U S A. 2018 Oct 30; 115(44): E10495–E10504. Published online 2018 Oct 17. doi: 10.1073/pnas.1810522115

PMCID:: PMC6217421

Abstract Article PDF–3.2M

Select item 512734814.

Motor neuron disease, TDP-43 pathology, and memory deficits in mice expressing ALS–FTD-linked UBQLN2 mutations

Nhat T. T. Le, Lydia Chang, Irina Kovlyagina, Polymnia Georgiou, Nathaniel Safren, Kerstin E. Braunstein, Mark D. Kvarta, Adam M. Van Dyke, Tara A. LeGates, Thomas Philips, Brett M. Morrison, Scott M. Thompson, Adam C. Puche, Todd D. Gould, Jeffrey D. Rothstein, Philip C. Wong, Mervyn J. Monteiro

Proc Natl Acad Sci U S A. 2016 Nov 22; 113(47): E7580–E7589. Published online 2016 Nov 9. doi: 10.1073/pnas.1608432113

Correction in:: Proc Natl Acad Sci U S A. 2025 Jan 14; 122(2): e2424914121.

PMCID:: PMC5127348

Abstract Article PDF–2.4M

Select item 501612215.

Amyloid Precursor Protein (APP) May Act as a Substrate and a Recognition Unit for CRL4^CRBN and Stub1 E3 Ligases Facilitating Ubiquitination of Proteins Involved in Presynaptic Functions and Neurodegeneration

Dolores Del Prete, Richard C. Rice, Anjali M. Rajadhyaksha, Luciano D'Adamio

J Biol Chem. 2016 Aug 12; 291(33): 17209–17227. Published online 2016 Jun 20. doi: 10.1074/jbc.M116.733626

PMCID:: PMC5016122

Abstract Article PDF–1.8M

Select item 500381616.

UBQLN2 Mediates Autophagy-Independent Protein Aggregate Clearance by the Proteasome

Roland Hjerpe, John S. Bett, Matthew J. Keuss, Alexandra Solovyova, Thomas G. McWilliams, Clare Johnson, Indrajit Sahu, Joby Varghese, Nicola Wood, Melanie Wightman, Georgina Osborne, Gillian P. Bates, Michael H. Glickman, Matthias Trost, Axel Knebel, Francesco Marchesi, Thimo Kurz

Cell. 2016 Aug 11; 166(4): 935–949. doi: 10.1016/j.cell.2016.07.001

PMCID:: PMC5003816

Abstract Article

Select item 464227617.

Differential recruitment of UBQLN2 to nuclear inclusions in the polyglutamine diseases HD and SCA3

Li Zeng, Bo Wang, Sean A Merillat, Eiko Minakawa, Matthew D Perkins, Biswarathan Ramani, Sara J. Tallaksen-Greene, Maria do Carmo Costa, Roger L. Albin, Henry L. Paulson

Neurobiol Dis. Author manuscript; available in PMC 2016 Oct 1.

Published in final edited form as: Neurobiol Dis. 2015 Oct; 82: 281–288. Published online 2015 Jun 30. doi: 10.1016/j.nbd.2015.06.017

PMCID:: PMC4642276

Abstract Article PDF–1.8M

Select item 462836118.

Ubiquilin-2 drives NF-κB activity and cytosolic TDP-43 aggregation in neuronal cells

Vincent Picher-Martel, Kallol Dutta, Daniel Phaneuf, Gen Sobue, Jean-Pierre Julien

Mol Brain. 2015; 8: 71. Published online 2015 Oct 31. doi: 10.1186/s13041-015-0162-6

PMCID:: PMC4628361

Abstract Article PDF–1.9M

Select item 449563919.

Viral expression of ALS-linked ubiquilin-2 mutants causes inclusion pathology and behavioral deficits in mice

Carolina Ceballos-Diaz, Awilda M. Rosario, Hyo-Jin Park, Paramita Chakrabarty, Amanda Sacino, Pedro E. Cruz, Zoe Siemienski, Nicolas Lara, Corey Moran, Natalia Ravelo, Todd E. Golde, Nikolaus R. McFarland

Mol Neurodegener. 2015; 10: 25. Published online 2015 Jul 8. doi: 10.1186/s13024-015-0026-7

PMCID:: PMC4495639

Abstract Article PDF–3.7M

Select item 404931020.

C9ORF72, implicated in amytrophic lateral sclerosis and frontotemporal dementia, regulates endosomal trafficking

Manal A. Farg, Vinod Sundaramoorthy, Jessica M. Sultana, Shu Yang, Rachel A.K. Atkinson, Vita Levina, Mark A. Halloran, Paul A. Gleeson, Ian P. Blair, Kai Y. Soo, Anna E. King, Julie D. Atkin

Hum Mol Genet. 2014 Jul 1; 23(13): 3579–3595. Published online 2014 Feb 18. doi: 10.1093/hmg/ddu068

Correction in:: Hum Mol Genet. 2017 Oct 15; 26(20): 4093–4094.

PMCID:: PMC4049310

Abstract Article PDF–1.8M

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