Mechanisms of TDP-43 Proteinopathy Onset and Propagation

av Chen, Han-Jou, Mitchell, Jacqueline C.
I publikationen Int J Mol Sci (2021)

Identification of a novel interaction of FUS and syntaphilin may explain synaptic and mitochondrial abnormalities caused by ALS mutations

av Salam, Shaakir, Tacconelli, Sara, Smith, Bradley N., Mitchell, Jacqueline C., Glennon, Elizabeth, Nikolaou, Nikolas, Houart, Corinne, Vance, Caroline
I publikationen Sci Rep (2021)

Mitochondrial abnormalities and disruption of the neuromuscular junction precede the clinical phenotype and motor neuron loss in hFUS(WT) transgenic mice

av So, Eva, Mitchell, Jacqueline C, Memmi, Caroline, Chennell, George, Vizcay-Barrena, Gema, Allison, Leanne, Shaw, Christopher E, Vance, Caroline
I publikationen Hum Mol Genet (2018)

Expression of the Neuronal Adaptor Protein X11α Protects Against Memory Dysfunction in a Transgenic Mouse Model of Alzheimer’s Disease

av Mitchell, Jacqueline C., Perkinton, Michael S., Yates, Darran M., Lau, Kwok-Fai, Rogelj, Boris, Miller, Christopher C.J., McLoughlin, Declan M.
Publicerad 2010

The heat shock response plays an important role in TDP-43 clearance: evidence for dysfunction in amyotrophic lateral sclerosis

av Chen, Han-Jou, Mitchell, Jacqueline C., Novoselov, Sergey, Miller, Jack, Nishimura, Agnes L., Scotter, Emma L., Vance, Caroline A., Cheetham, Michael E., Shaw, Christopher E.
I publikationen Brain (2016)

X11β rescues memory and long-term potentiation deficits in Alzheimer's disease APPswe Tg2576 mice

av Mitchell, Jacqueline C., Ariff, Belall B., Yates, Darran M., Lau, Kwok-Fai, Perkinton, Michael S., Rogelj, Boris, Stephenson, John D., Miller, Christopher C.J., McLoughlin, Declan M.
Publicerad 2009

Differential roles of the ubiquitin proteasome system and autophagy in the clearance of soluble and aggregated TDP-43 species

av Scotter, Emma L., Vance, Caroline, Nishimura, Agnes L., Lee, Youn-Bok, Chen, Han-Jou, Urwin, Hazel, Sardone, Valentina, Mitchell, Jacqueline C., Rogelj, Boris, Rubinsztein, David C., Shaw, Christopher E.
Publicerad 2014

Tauopathy induced by low level expression of a human brain-derived tau fragment in mice is rescued by phenylbutyrate

av Bondulich, Marie K., Guo, Tong, Meehan, Christopher, Manion, John, Rodriguez Martin, Teresa, Mitchell, Jacqueline C., Hortobagyi, Tibor, Yankova, Natalia, Stygelbout, Virginie, Brion, Jean-Pierre, Noble, Wendy, Hanger, Diane P.
I publikationen Brain (2016)

ALS mutant FUS disrupts nuclear localization and sequesters wild-type FUS within cytoplasmic stress granules

av Vance, Caroline, Scotter, Emma L., Nishimura, Agnes L., Troakes, Claire, Mitchell, Jacqueline C., Kathe, Claudia, Urwin, Hazel, Manser, Catherine, Miller, Christopher C., Hortobágyi, Tibor, Dragunow, Mike, Rogelj, Boris, Shaw, Christopher E.
Publicerad 2013

Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion

av Mitchell, Jacqueline C., McGoldrick, Philip, Vance, Caroline, Hortobagyi, Tibor, Sreedharan, Jemeen, Rogelj, Boris, Tudor, Elizabeth L., Smith, Bradley N., Klasen, Christian, Miller, Christopher C. J., Cooper, Jonathan D., Greensmith, Linda, Shaw, Christopher E.
Publicerad 2012

Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS

av Mitchell, Jacqueline C, Constable, Remy, So, Eva, Vance, Caroline, Scotter, Emma, Glover, Leanne, Hortobagyi, Tibor, Arnold, Eveline S., Ling, Shuo-Chien, McAlonis, Melissa, Da Cruz, Sandrine, Polymenidou, Magda, Tessarolo, Lino, Cleveland, Don W, Shaw, Christopher E
I publikationen Acta Neuropathol Commun (2015)

ALS/FTD‐associated FUS activates GSK‐3β to disrupt the VAPB–PTPIP51 interaction and ER–mitochondria associations

av Stoica, Radu, Paillusson, Sébastien, Gomez‐Suaga, Patricia, Mitchell, Jacqueline C, Lau, Dawn HW, Gray, Emma H, Sancho, Rosa M, Vizcay‐Barrena, Gema, De Vos, Kurt J, Shaw, Christopher E, Hanger, Diane P, Noble, Wendy, Miller, Christopher CJ
I publikationen EMBO Rep (2016)

ER–mitochondria associations are regulated by the VAPB–PTPIP51 interaction and are disrupted by ALS/FTD-associated TDP-43

av Stoica, Radu, De Vos, Kurt J., Paillusson, Sébastien, Mueller, Sarah, Sancho, Rosa M., Lau, Kwok-Fai, Vizcay-Barrena, Gema, Lin, Wen-Lang, Xu, Ya-Fei, Lewis, Jada, Dickson, Dennis W., Petrucelli, Leonard, Mitchell, Jacqueline C., Shaw, Christopher E., Miller, Christopher C. J.
Publicerad 2014

Novel mutations support a role for Profilin 1 in the pathogenesis of ALS

av Smith, Bradley N., Vance, Caroline, Scotter, Emma L., Troakes, Claire, Wong, Chun Hao, Topp, Simon, Maekawa, Satomi, King, Andrew, Mitchell, Jacqueline C., Lund, Karan, Al-Chalabi, Ammar, Ticozzi, Nicola, Silani, Vincenzo, Sapp, Peter, Brown, Robert H., Landers, John E., Al-Sarraj, Safa, Shaw, Christopher E.
I publikationen Neurobiol Aging (2015)

Mutations in the vesicular trafficking protein annexin A11 are associated with amyotrophic lateral sclerosis

av Smith, Bradley N., Topp, Simon D., Fallini, Claudia, Shibata, Hideki, Chen, Han-Jou, Troakes, Claire, King, Andrew, Ticozzi, Nicola, Kenna, Kevin P., Soragia-Gkazi, Athina, Miller, Jack W., Sato, Akane, Dias, Diana Marques, Jeon, Maryangel, Vance, Caroline, Wong, Chun Hao, de Majo, Martina, Kattuah, Wejdan, Mitchell, Jacqueline C., Scotter, Emma L., Parkin, Nicholas W., Sapp, Peter C., Nolan, Matthew, Nestor, Peter J., Simpson, Michael, Weale, Michael, Lek, Monkel, Baas, Frank, de Jong, J. M. Vianney, ten Asbroek, Anneloor L. M. A., Redondo, Alberto Garcia, Esteban-Pérez, Jesús, Tiloca, Cinzia, Verde, Federico, Duga, Stefano, Leigh, Nigel, Pall, Hardev, Morrison, Karen E., Al-Chalabi, Ammar, Shaw, Pamela J., Kirby, Janine, Turner, Martin R., Talbot, Kevin, Hardiman, Orla, Glass, Jonathan D., de Belleroche, Jacqueline, Maki, Masatoshi, Moss, Stephen E., Miller, Christopher, Gellera, Cinzia, Ratti, Antonia, Al-Sarraj, Safa, Brown, Robert H., Silani, Vincenzo, Landers, John E., Shaw, Christopher E.
I publikationen Sci Transl Med (2017)