Axonal transport of TDP-43 mRNA granules is impaired by ALS-causing mutations von Alami, Nael H, Smith, Rebecca B, Carrasco, Monica A, Williams, Luis A, Winborn, Christina S, Han, Steve S W, Kiskinis, Evangelos, Winborn, Brett, Freibaum, Brian D, Kanagaraj, Anderson, Clare, Alison J, Badders, Nisha M, Bilican, Bilada, Chaum, Edward, Chandran, Siddharthan, Shaw, Christopher E, Eggan, Kevin C, Maniatis, Tom, Taylor, J Paul

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Hexanucleotide Repeats in ALS/FTD Form Length-Dependent RNA Foci, Sequester RNA Binding Proteins, and Are Neurotoxic von Lee, Youn-Bok, Chen, Han-Jou, Peres, João N., Gomez-Deza, Jorge, Attig, Jan, Štalekar, Maja, Troakes, Claire, Nishimura, Agnes L., Scotter, Emma L., Vance, Caroline, Adachi, Yoshitsugu, Sardone, Valentina, Miller, Jack W., Smith, Bradley N., Gallo, Jean-Marc, Ule, Jernej, Hirth, Frank, Rogelj, Boris, Houart, Corinne, Shaw, Christopher E.

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

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RRM adjacent TARDBP mutations disrupt RNA binding and enhance TDP-43 proteinopathy von Chen, Han-Jou, Topp, Simon D, Hui, Ho Sang, Zacco, Elsa, Katarya, Malvika, McLoughlin, Conor, King, Andrew, Smith, Bradley N, Troakes, Claire, Pastore, Annalisa, Shaw, Christopher E

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p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD and MND/ALS von Al-Sarraj, Safa, King, Andrew, Troakes, Claire, Smith, Bradley, Maekawa, Satomi, Bodi, Istvan, Rogelj, Boris, Al-Chalabi, Ammar, Hortobágyi, Tibor, Shaw, Christopher E.

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

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Characterizing the RNA targets and position-dependent splicing regulation by TDP-43 von Ule, Jernej, Tollervey, James R, Curk, Toma, Rogelj, Boris, Briese, Michael, Cereda, Matteo, Kayikci, Melis, König, Julian, Hortobágyi, Tibor, Nishimura, Agnes L, upunski, Vera, Patani, Rickie, Chandran, Siddharthan, Rot, Gregor, Zupan, Bla, Shaw, Christopher E

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Mutant C9orf72 human iPSC‐derived astrocytes cause non‐cell autonomous motor neuron pathophysiology von Zhao, Chen, Devlin, Anna‐Claire, Chouhan, Amit K., Selvaraj, Bhuvaneish T., Stavrou, Maria, Burr, Karen, Brivio, Veronica, He, Xin, Mehta, Arpan R., Story, David, Shaw, Christopher E., Dando, Owen, Hardingham, Giles E., Miles, Gareth B., Chandran, Siddharthan

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

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Astrocyte pathology and the absence of non-cell autonomy in an induced pluripotent stem cell model of TDP-43 proteinopathy von Serio, Andrea, Bilican, Bilada, Barmada, Sami J., Ando, Dale Michael, Zhao, Chen, Siller, Rick, Burr, Karen, Haghi, Ghazal, Story, David, Nishimura, Agnes Lumi, Carrasco, Monica A., Phatnani, Hemali P., Shum, Carole, Wilmut, Ian, Maniatis, Tom, Shaw, Christopher E., Finkbeiner, Steven, Chandran, Siddharthan

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ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43 von Arnold, Eveline S, Ling, Shuo-Chien, Huelga, Stephanie C, Lagier-Tourenne, Clotilde, Polymenidou, Magdalini, Ditsworth, Dara, Kordasiewicz, Holly B, McAlonis-Downes, Melissa, Platoshyn, Oleksandr, Parone, Philippe A, Da Cruz, Sandrine, Clutario, Kevin M, Swing, Debbie, Tessarollo, Lino, Marsala, Martin, Shaw, Christopher E, Yeo, Gene W, Cleveland, Don W

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

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Analysis of amyotrophic lateral sclerosis as a multistep process: a population-based modelling study von Al-Chalabi, Ammar, Prof, Calvo, Andrea, MD, Chio, Adriano, Prof, Colville, Shuna, MPH, Ellis, Cathy M, PhD, Hardiman, Orla, Prof, Heverin, Mark, MSc, Howard, Robin S, PhD, Huisman, Mark H B, MD, Keren, Noa, MBBS, Leigh, P Nigel, Prof, Mazzini, Letizia, MD, Mora, Gabriele, MD, Orrell, Richard W, MD, Rooney, James, MSc, Scott, Kirsten M, MBBS, Scotton, William J, MBioch, Seelen, Meinie, MD, Shaw, Christopher E, Prof, Sidle, Katie S, PhD, Swingler, Robert, MD, Tsuda, Miho, MBChB, Veldink, Jan H, Prof, Visser, Anne E, MD, van den Berg, Leonard H, Prof, Pearce, Neil, Prof

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FUS-SMN Protein Interactions Link the Motor Neuron Diseases ALS and SMA von Yamazaki, Tomohiro, Chen, Shi, Yu, Yong, Yan, Biao, Haertlein, Tyler C., Carrasco, Monica A., Tapia, Juan C., Zhai, Bo, Das, Rita, Lalancette-Hebert, Melanie, Sharma, Aarti, Chandran, Siddharthan, Sullivan, Gareth, Nishimura, Agnes Lumi, Shaw, Christopher E., Gygi, Steve P., Shneider, Neil A., Maniatis, Tom, Reed, Robin

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Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6 von Vance, Caroline, Rogelj, Boris, Hortobágyi, Tibor, De Vos, Kurt J, Nishimura, Agnes Lumi, Sreedharan, Jemeen, Hu, Xun, Smith, Bradley, Ruddy, Deborah, Wright, Paul, Ganesalingam, Jeban, Williams, Kelly L, Tripathi, Vineeta, Al-Saraj, Safa, Al-Chalabi, Ammar, Leigh, P. Nigel, Blair, Ian P, Nicholson, Garth, de Belleroche, Jackie, Gallo, Jean-Marc, Miller, Christopher C, Shaw, Christopher E

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A comprehensive analysis of rare genetic variation in amyotrophic lateral sclerosis in the UK von Morgan, Sarah, Shatunov, Aleksey, Sproviero, William, Jones, Ashley R, Shoai, Maryam, Hughes, Deborah, Al Khleifat, Ahmad, Malaspina, Andrea, Morrison, Karen E, Shaw, Pamela J, Shaw, Christopher E, Sidle, Katie, Orrell, Richard W, Fratta, Pietro, Hardy, John, Pittman, Alan, Al-Chalabi, Ammar

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CYLD is a causative gene for frontotemporal dementia - amyotrophic lateral sclerosis von Dobson-Stone, Carol, Hallupp, Marianne, Shahheydari, Hamideh, Ragagnin, Audrey M G, Chatterton, Zac, Carew-Jones, Francine, Shepherd, Claire E, Stefen, Holly, Paric, Esmeralda, Fath, Thomas, Thompson, Elizabeth M, Blumbergs, Peter, Short, Cathy L, Field, Colin D, Panegyres, Peter K, Hecker, Jane, Nicholson, Garth, Shaw, Alex D, Fullerton, Janice M, Luty, Agnes A, Schofield, Peter R, Brooks, William S, Rajan, Neil, Bennett, Mark F, Bahlo, Melanie, Landers, John E, Piguet, Olivier, Hodges, John R, Halliday, Glenda M, Topp, Simon D, Smith, Bradley N, Shaw, Christopher E, McCann, Emily, Fifita, Jennifer A, Williams, Kelly L, Atkin, Julie D, Blair, Ian P, Kwok, John B

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TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis von Sreedharan, Jemeen, Blair, Ian P, Tripathi, Vineeta B, Hu, Xun, Vance, Caroline, Rogelj, Boris, Ackerley, Steven, Durnall, Jennifer C, Williams, Kelly L, Buratti, Emanuele, Baralle, Francisco, de Belleroche, Jacqueline, Mitchell, J. Douglas, Leigh, P. Nigel, Al-Chalabi, Ammar, Miller, Christopher C, Nicholson, Garth, Shaw, Christopher E

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Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability von Bilican, Bilada, Serio, Andrea, Barmada, Sami J, Nishimura, Agnes Lumi, Sullivan, Gareth J, Carrasco, Monica, Phatnani, Hemali P, Puddifoot, Clare A, Story, David, Fletcher, Judy, Park, In-Hyun, Friedman, Brad A, Daley, George Q, Wyllie, David J. A, Hardingham, Giles E, Wilmut, Ian, Finkbeiner, Steven, Maniatis, Tom, Shaw, Christopher E, Chandran, Siddharthan

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Cytoplasmic TDP-43 is involved in cell fate during stress recovery von Lee, Youn-Bok, Scotter, Emma L, Lee, Do-Young, Troakes, Claire, Mitchell, Jacqueline, Rogelj, Boris, Gallo, Jean-Marc, Shaw, Christopher E

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