Search Results - "McCluskey, Leo"

Stages of pTDP-43 pathology in amyotrophic lateral sclerosis by Brettschneider, Johannes, Del Tredici, Kelly, Toledo, Jon B., Robinson, John L., Irwin, David J., Grossman, Murray, Suh, EunRan, Van Deerlin, Vivianna M., Wood, Elisabeth M., Baek, Young, Kwong, Linda, Lee, Edward B., Elman, Lauren, McCluskey, Leo, Fang, Lubin, Feldengut, Simone, Ludolph, Albert C., Lee, Virginia M.-Y., Braak, Heiko, Trojanowski, John Q.

“…Objective To see whether the distribution patterns of phosphorylated 43kDa TAR DNA‐binding protein (pTDP‐43) intraneuronal inclusions in amyotrophic lateral…”
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Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis by Neumann, Manuela, Sampathu, Deepak M, Kwong, Linda K, Truax, Adam C, Micsenyi, Matthew C, Chou, Thomas T, Bruce, Jennifer, Schuck, Theresa, Grossman, Murray, Clark, Christopher M, McCluskey, Leo F, Miller, Bruce L, Masliah, Eliezer, Mackenzie, Ian R, Feldman, Howard, Feiden, Wolfgang, Kretzschmar, Hans A, Trojanowski, John Q, Lee, Virginia M.-Y

“…Ubiquitin-positive, tau- and α-synuclein-negative inclusions are hallmarks of frontotemporal lobar degeneration with ubiquitin-positive inclusions and…”
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Circulating brain-enriched microRNAs as novel biomarkers for detection and differentiation of neurodegenerative diseases by Sheinerman, Kira S, Toledo, Jon B, Tsivinsky, Vladimir G, Irwin, David, Grossman, Murray, Weintraub, Daniel, Hurtig, Howard I, Chen-Plotkin, Alice, Wolk, David A, McCluskey, Leo F, Elman, Lauren B, Trojanowski, John Q, Umansky, Samuil R

“…Minimally invasive specific biomarkers of neurodegenerative diseases (NDs) would facilitate patient selection and disease progression monitoring. We describe…”
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Poly(GP) proteins are a useful pharmacodynamic marker for C9ORF72 -associated amyotrophic lateral sclerosis by Gendron, Tania F, Chew, Jeannie, Stankowski, Jeannette N, Hayes, Lindsey R, Zhang, Yong-Jie, Prudencio, Mercedes, Carlomagno, Yari, Daughrity, Lillian M, Jansen-West, Karen, Perkerson, Emilie A, O'Raw, Aliesha, Cook, Casey, Pregent, Luc, Belzil, Veronique, van Blitterswijk, Marka, Tabassian, Lilia J, Lee, Chris W, Yue, Mei, Tong, Jimei, Song, Yuping, Castanedes-Casey, Monica, Rousseau, Linda, Phillips, Virginia, Dickson, Dennis W, Rademakers, Rosa, Fryer, John D, Rush, Beth K, Pedraza, Otto, Caputo, Ana M, Desaro, Pamela, Palmucci, Carla, Robertson, Amelia, Heckman, Michael G, Diehl, Nancy N, Wiggs, Edythe, Tierney, Michael, Braun, Laura, Farren, Jennifer, Lacomis, David, Ladha, Shafeeq, Fournier, Christina N, McCluskey, Leo F, Elman, Lauren B, Toledo, Jon B, McBride, Jennifer D, Tiloca, Cinzia, Morelli, Claudia, Poletti, Barbara, Solca, Federica, Prelle, Alessandro, Wuu, Joanne, Jockel-Balsarotti, Jennifer, Rigo, Frank, Ambrose, Christine, Datta, Abhishek, Yang, Weixing, Raitcheva, Denitza, Antognetti, Giovanna, McCampbell, Alexander, Van Swieten, John C, Miller, Bruce L, Boxer, Adam L, Brown, Robert H, Bowser, Robert, Miller, Timothy M, Trojanowski, John Q, Grossman, Murray, Berry, James D, Hu, William T, Ratti, Antonia, Traynor, Bryan J, Disney, Matthew D, Benatar, Michael, Silani, Vincenzo, Glass, Jonathan D, Floeter, Mary Kay, Rothstein, Jeffrey D, Boylan, Kevin B, Petrucelli, Leonard

“…There is no effective treatment for amyotrophic lateral sclerosis (ALS), a devastating motor neuron disease. However, discovery of a G C repeat expansion in…”
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Cerebrospinal fluid neurogranin concentration in neurodegeneration: relation to clinical phenotypes and neuropathology by Portelius, Erik, Olsson, Bob, Höglund, Kina, Cullen, Nicholas C., Kvartsberg, Hlin, Andreasson, Ulf, Zetterberg, Henrik, Sandelius, Åsa, Shaw, Leslie M., Lee, Virginia M. Y., Irwin, David J., Grossman, Murray, Weintraub, Daniel, Chen-Plotkin, Alice, Wolk, David A., McCluskey, Leo, Elman, Lauren, McBride, Jennifer, Toledo, Jon B., Trojanowski, John Q., Blennow, Kaj

“…Neurogranin (Ng) is a post-synaptic protein that previously has been shown to be a biomarker for synaptic function when measured in cerebrospinal fluid (CSF)…”
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Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study by Majounie, Elisa, PhD, Renton, Alan E, PhD, Mok, Kin, MSc, Dopper, Elise GP, Waite, Adrian, PhD, Rollinson, Sara, PhD, Chiò, Adriano, MD, Restagno, Gabriella, MD, Nicolaou, Nayia, MSc, Simon-Sanchez, Javier, PhD, van Swieten, John C, Prof, Abramzon, Yevgeniya, Johnson, Janel O, PhD, Sendtner, Michael, Prof, Pamphlett, Roger, MD, Orrell, Richard W, MD, Mead, Simon, MD, Sidle, Katie C, MD, Houlden, Henry, Prof, Rohrer, Jonathan D, MD, Morrison, Karen E, Prof, Pall, Hardev, MD, Talbot, Kevin, Prof, Ansorge, Olaf, MD, Hernandez, Dena G, MSc, Arepalli, Sampath, MS, Sabatelli, Mario, MD, Mora, Gabriele, MD, Corbo, Massimo, MD, Giannini, Fabio, MD, Calvo, Andrea, MD, Englund, Elisabet, MD, Borghero, Giuseppe, MD, Floris, Gian Luca, MD, Remes, Anne M, Prof, Laaksovirta, Hannu, MD, McCluskey, Leo, MD, Trojanowski, John Q, Prof, Van Deerlin, Vivianna M, MD, Schellenberg, Gerard D, Prof, Nalls, Michael A, PhD, Drory, Vivian E, MD, Lu, Chin-Song, Prof, Yeh, Tu-Hsueh, MD, Ishiura, Hiroyuki, MD, Takahashi, Yuji, MD, Tsuji, Shoji, Prof, Le Ber, Isabelle, MD, Brice, Alexis, Prof, Drepper, Carsten, PhD, Williams, Nigel, PhD, Kirby, Janine, PhD, Shaw, Pamela, Prof, Hardy, John, Prof, Tienari, Pentti J, MD, Heutink, Peter, Prof, Morris, Huw R, MD, Pickering-Brown, Stuart, Prof, Traynor, Bryan J, Dr

“…Summary Background We aimed to accurately estimate the frequency of a hexanucleotide repeat expansion in C9orf72 that has been associated with a large…”
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Efficacy of minocycline in patients with amyotrophic lateral sclerosis: a phase III randomised trial by Gordon, Paul H, MD, Moore, Dan H, PhD, Miller, Robert G, MD, Florence, Julaine M, DPT, Verheijde, Joseph L, PhD, Doorish, Carolyn, BA, Hilton, Joan F, ScD, Spitalny, G Mark, MA, MacArthur, Robert B, PhD, Mitsumoto, Hiroshi, MD, Neville, Hans E, MD, Boylan, Kevin, MD, Mozaffar, Tahseen, MD, Belsh, Jerry M, MD, Ravits, John, MD, Bedlack, Richard S, MD, Graves, Michael C, MD, McCluskey, Leo F, MD, Barohn, Richard J, MD, Tandan, Rup, MD

“…Summary Background Minocycline has anti-apoptotic and anti-inflammatory effects in vitro, and extends survival in mouse models of some neurological conditions…”
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TDP-43 pathology and neuronal loss in amyotrophic lateral sclerosis spinal cord by Brettschneider, Johannes, Arai, Kimihito, Del Tredici, Kelly, Toledo, Jon B., Robinson, John L., Lee, Edward B., Kuwabara, Satoshi, Shibuya, Kazumoto, Irwin, David J., Fang, Lubin, Van Deerlin, Vivianna M., Elman, Lauren, McCluskey, Leo, Ludolph, Albert C., Lee, Virginia M.-Y., Braak, Heiko, Trojanowski, John Q.

“…We examined the phosphorylated 43-kDa TAR DNA-binding protein (pTDP-43) inclusions as well as neuronal loss in full-length spinal cords and five selected…”
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Defining SOD1 ALS natural history to guide therapeutic clinical trial design by Bali, Taha, Self, Wade, Liu, Jingxia, Siddique, Teepu, Wang, Leo H, Bird, Thomas D, Ratti, Elena, Atassi, Nazem, Boylan, Kevin B, Glass, Jonathan D, Maragakis, Nicholas J, Caress, James B, McCluskey, Leo F, Appel, Stanley H, Wymer, James P, Gibson, Summer, Zinman, Lorne, Mozaffar, Tahseen, Callaghan, Brian, McVey, April L, Jockel-Balsarotti, Jennifer, Allred, Peggy, Fisher, Elena R, Lopate, Glenn, Pestronk, Alan, Cudkowicz, Merit E, Miller, Timothy M

“…ImportanceUnderstanding the natural history of familial amyotrophic lateral sclerosis (ALS) caused by SOD1 mutations (ALSSOD1) will provide key information for…”
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Microglial activation correlates with disease progression and upper motor neuron clinical symptoms in amyotrophic lateral sclerosis by Brettschneider, Johannes, Toledo, Jon B, Van Deerlin, Vivianna M, Elman, Lauren, McCluskey, Leo, Lee, Virginia M-Y, Trojanowski, John Q

“…We evaluated clinicopathological correlates of upper motor neuron (UMN) damage in amyotrophic lateral sclerosis (ALS), and analyzed if the presence of the…”
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Characterization of Parkinson's disease using blood-based biomarkers: A multicohort proteomic analysis by Posavi, Marijan, Diaz-Ortiz, Maria, Liu, Benjamine, Swanson, Christine R, Skrinak, R Tyler, Hernandez-Con, Pilar, Amado, Defne A, Fullard, Michelle, Rick, Jacqueline, Siderowf, Andrew, Weintraub, Daniel, McCluskey, Leo, Trojanowski, John Q, Dewey, Jr, Richard B, Huang, Xuemei, Chen-Plotkin, Alice S

“…Parkinson's disease (PD) is a progressive neurodegenerative disease affecting about 5 million people worldwide with no disease-modifying therapies. We sought…”
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Hypermethylation of repeat expanded C9orf72 is a clinical and molecular disease modifier by Russ, Jenny, Liu, Elaine Y., Wu, Kathryn, Neal, Donald, Suh, EunRan, Irwin, David J., McMillan, Corey T., Harms, Matthew B., Cairns, Nigel J., Wood, Elisabeth M., Xie, Sharon X., Elman, Lauren, McCluskey, Leo, Grossman, Murray, Van Deerlin, Vivianna M., Lee, Edward B.

“…C9orf72 promoter hypermethylation inhibits the accumulation of pathologies which have been postulated to be neurotoxic. We tested here whether C9orf72…”
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Cognitive decline and reduced survival in C9orf72 expansion frontotemporal degeneration and amyotrophic lateral sclerosis by Irwin, David J, McMillan, Corey T, Brettschneider, Johannes, Libon, David J, Powers, John, Rascovsky, Katya, Toledo, Jon B, Boller, Ashley, Bekisz, Jonathan, Chandrasekaran, Keerthi, Wood, Elisabeth McCarty, Shaw, Leslie M, Woo, John H, Cook, Philip A, Wolk, David A, Arnold, Steven E, Van Deerlin, Vivianna M, McCluskey, Leo F, Elman, Lauren, Lee, Virginia M-Y, Trojanowski, John Q, Grossman, Murray

“…Background Significant heterogeneity in clinical features of frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS) cases with the…”
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Plasma multianalyte profiling in mild cognitive impairment and Alzheimer disease by HU, William T, HOLTZMAN, David M, PICKERING, Eve, KUHN, Max, YU CHEN, VAN DEERLIN, Vivianna M, MCCLUSKEY, Leo, ELMAN, Lauren, KARLAWISH, Jason, CHEN-PLOTKIN, Alice, HURTIG, Howard I, SIDEROWF, Andrew, FAGAN, Anne M, SWENSON, Frank, LEE, Virginia M.-Y, MORRIS, John C, TROJANOWSKI, John Q, SOARES, Holly, SHAW, Leslie M, PERRIN, Richard, ARNOLD, Steven E, GROSSMAN, Murray, CHENGJIE XIONG, CRAIG-SCHAPIRO, Rebecca, CLARK, Christopher M

“…While plasma biomarkers have been proposed to aid in the clinical diagnosis of Alzheimer disease (AD), few biomarkers have been validated in independent…”
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Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosis by COUTHOUIS, Julien, HART, Michael P, KIM, Cecilia E, FRACKELTON, Edward C, SOLSKI, Jennifer A, WILLIAMS, Kelly L, CLAY-FALCONE, Dana, ELMAN, Lauren, MCCLUSKEY, Leo, GREENE, Robert, HAKONARSON, Hakon, KALB, Robert G, ERION, Renske, LEE, Virginia M. Y, TROJANOWSKI, John Q, NICHOLSON, Garth A, BLAIR, Ian P, BONINI, Nancy M, VAN DEERLIN, Vivianna M, MOURELATOS, Zissimos, SHORTER, James, GITLER, Aaron D, KING, Oliver D, DIAZ, Zamia, NAKAYA, Tadashi, IBRAHIM, Fadia, KIM, Hyung-Jun, MOJSILOVIC-PETROVIC, Jelena, PANOSSIAN, Saarene

“…Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease affecting motor neurons. Mutations in related RNA-binding proteins TDP-43, FUS/TLS and…”
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TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: a genetic and histopathological analysis by Van Deerlin, Vivianna M, MD, Leverenz, James B, MD, Bekris, Lynn M, PhD, Bird, Thomas D, MD, Yuan, Wuxing, MS, Elman, Lauren B, MD, Clay, Dana, MS, Wood, Elisabeth McCarty, MS, Chen-Plotkin, Alice S, MD, Martinez-Lage, Maria, MD, Steinbart, Ellen, MA, McCluskey, Leo, MD, Grossman, Murray, MD, Neumann, Manuela, MD, Wu, I-Lin, MS, Yang, Wei-Shiung, MD, Kalb, Robert, MD, Galasko, Douglas R, MD, Montine, Thomas J, MD, Trojanowski, John Q, MD, Lee, Virginia M-Y, PhD, Schellenberg, Gerard D, PhD, Yu, Chang-En, PhD

“…Summary Background TDP-43 is a major component of the ubiquitinated inclusions that characterise amyotrophic lateral sclerosis (ALS) and frontotemporal lobar…”
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Phosphorylated tau as a candidate biomarker for amyotrophic lateral sclerosis by Grossman, Murray, Elman, Lauren, McCluskey, Leo, McMillan, Corey T, Boller, Ashley, Powers, John, Rascovsky, Katya, Hu, William, Shaw, Les, Irwin, David J, Lee, Virginia M-Y, Trojanowski, John Q

“…An increasingly varied clinical spectrum of cases with amyotrophic lateral sclerosis (ALS) has been identified, and objective criteria for clinical trial…”
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Pattern of ubiquilin pathology in ALS and FTLD indicates presence of C9ORF72 hexanucleotide expansion by Brettschneider, Johannes, Van Deerlin, Vivianna M., Robinson, John L., Kwong, Linda, Lee, Edward B., Ali, Yousuf O., Safren, Nathaniel, Monteiro, Mervyn J., Toledo, Jon B., Elman, Lauren, McCluskey, Leo, Irwin, David J., Grossman, Murray, Molina-Porcel, Laura, Lee, Virginia M.-Y., Trojanowski, John Q.

“…C9ORF72 -hexanucleotide repeat expansions and ubiquilin-2 ( UBQLN2 ) mutations are recently identified genetic markers in amyotrophic lateral sclerosis (ALS)…”
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UNC13A polymorphism contributes to frontotemporal disease in sporadic amyotrophic lateral sclerosis by Placek, Katerina, Baer, G. Michael, Elman, Lauren, McCluskey, Leo, Hennessy, Laura, Ferraro, Pilar M., Lee, Edward B., Lee, Virginia M.Y., Trojanowski, John Q., Van Deerlin, Vivianna M., Grossman, Murray, Irwin, David J., McMillan, Corey T.

“…The majority (90%–95%) of amyotrophic lateral sclerosis (ALS) is sporadic, and ∼50% of patients develop symptoms of frontotemporal degeneration (FTD)…”
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Enrichment of C-Terminal Fragments in TAR DNA-Binding Protein-43 Cytoplasmic Inclusions in Brain but not in Spinal Cord of Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis by Igaz, Lionel M, Kwong, Linda K, Xu, Yan, Truax, Adam C, Uryu, Kunihiro, Neumann, Manuela, Clark, Christopher M, Elman, Lauren B, Miller, Bruce L, Grossman, Murray, McCluskey, Leo F, Trojanowski, John Q, Lee, Virginia M.-Y

“…TAR DNA-binding protein (TDP-43) has been recently described as a major pathological protein in both frontotemporal dementia with ubiquitin-positive inclusions…”
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