Search Results - "Shneider, A."

Antisense oligonucleotide silencing of FUS expression as a therapeutic approach in amyotrophic lateral sclerosis by Korobeynikov, Vladislav A., Lyashchenko, Alexander K., Blanco-Redondo, Beatriz, Jafar-Nejad, Paymaan, Shneider, Neil A.

“…Fused in sarcoma (FUS) is an RNA-binding protein that is genetically and pathologically associated with rare and aggressive forms of amyotrophic lateral…”
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FUS affects circular RNA expression in murine embryonic stem cell-derived motor neurons by Errichelli, Lorenzo, Dini Modigliani, Stefano, Laneve, Pietro, Colantoni, Alessio, Legnini, Ivano, Capauto, Davide, Rosa, Alessandro, De Santis, Riccardo, Scarfò, Rebecca, Peruzzi, Giovanna, Lu, Lei, Caffarelli, Elisa, Shneider, Neil A., Morlando, Mariangela, Bozzoni, Irene

“…The RNA-binding protein FUS participates in several RNA biosynthetic processes and has been linked to the pathogenesis of amyotrophic lateral sclerosis (ALS)…”
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ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function by Sharma, Aarti, Lyashchenko, Alexander K., Lu, Lei, Nasrabady, Sara Ebrahimi, Elmaleh, Margot, Mendelsohn, Monica, Nemes, Adriana, Tapia, Juan Carlos, Mentis, George Z., Shneider, Neil A.

“…Mutations in FUS cause amyotrophic lateral sclerosis (ALS), including some of the most aggressive, juvenile-onset forms of the disease. FUS loss-of-function…”
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Gamma motor neurons survive and exacerbate alpha motor neuron degeneration in ALS by Lalancette-Hebert, Melanie, Sharma, Aarti, Lyashchenko, Alexander K., Shneider, Neil A.

“…The molecular and cellular basis of selective motor neuron (MN) vulnerability in amyotrophic lateral sclerosis (ALS) is not known. In genetically distinct…”
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Antisense Proline-Arginine RAN Dipeptides Linked to C9ORF72-ALS/FTD Form Toxic Nuclear Aggregates that Initiate In Vitro and In Vivo Neuronal Death by Wen, Xinmei, Tan, Wenzhi, Westergard, Thomas, Krishnamurthy, Karthik, Markandaiah, Shashirekha S., Shi, Yingxiao, Lin, Shaoyu, Shneider, Neil A., Monaghan, John, Pandey, Udai B., Pasinelli, Piera, Ichida, Justin K., Trotti, Davide

“…Expanded GGGGCC (G4C2) nucleotide repeats within the C9ORF72 gene are the most common genetic mutation associated with both amyotrophic lateral sclerosis (ALS)…”
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Mutant TDP-43 Causes Early-Stage Dose-Dependent Motor Neuron Degeneration in a TARDBP Knockin Mouse Model of ALS by Ebstein, Sarah Y., Yagudayeva, Ilona, Shneider, Neil A.

“…Rare mutations in TARDBP, the gene encoding TDP-43, cause amyotrophic lateral sclerosis (ALS), and TDP-43 pathology is seen in a large majority of ALS…”
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The C9ORF72 GGGGCC expansion forms RNA G-quadruplex inclusions and sequesters hnRNP H to disrupt splicing in ALS brains by Conlon, Erin G, Lu, Lei, Sharma, Aarti, Yamazaki, Takashi, Tang, Timothy, Shneider, Neil A, Manley, James L

“…An expanded GGGGCC hexanucleotide in (C9) is the most frequent known cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). It has…”
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ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function by Murakami, Tetsuro, Qamar, Seema, Lin, Julie Qiaojin, Schierle, Gabriele S. Kaminski, Rees, Eric, Miyashita, Akinori, Costa, Ana R., Dodd, Roger B., Chan, Fiona T.S., Michel, Claire H., Kronenberg-Versteeg, Deborah, Li, Yi, Yang, Seung-Pil, Wakutani, Yosuke, Meadows, William, Ferry, Rodylyn Rose, Dong, Liang, Tartaglia, Gian Gaetano, Favrin, Giorgio, Lin, Wen-Lang, Dickson, Dennis W., Zhen, Mei, Ron, David, Schmitt-Ulms, Gerold, Fraser, Paul E., Shneider, Neil A., Holt, Christine, Vendruscolo, Michele, Kaminski, Clemens F., St George-Hyslop, Peter

“…The mechanisms by which mutations in FUS and other RNA binding proteins cause ALS and FTD remain controversial. We propose a model in which low-complexity (LC)…”
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The ALS-associated proteins FUS and TDP-43 function together to affect Drosophila locomotion and life span by Wang, Ji-Wu, Brent, Jonathan R, Tomlinson, Andrew, Shneider, Neil A, McCabe, Brian D

“…The fatal adult motor neuron disease amyotrophic lateral sclerosis (ALS) shares some clinical and pathological overlap with frontotemporal dementia (FTD), an…”
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Hypoexcitability precedes denervation in the large fast-contracting motor units in two unrelated mouse models of ALS by Martínez-Silva, María de Lourdes, Imhoff-Manuel, Rebecca D, Sharma, Aarti, Heckman, C J, Shneider, Neil A, Roselli, Francesco, Zytnicki, Daniel, Manuel, Marin

“…Hyperexcitability has been suggested to contribute to motoneuron degeneration in amyotrophic lateral sclerosis (ALS). If this is so, and given that the…”
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FUS-SMN Protein Interactions Link the Motor Neuron Diseases ALS and SMA by 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

“…Mutations in the RNA binding protein FUS cause amyotrophic lateral sclerosis (ALS), a fatal adult motor neuron disease. Decreased expression of SMN causes the…”
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Amyotrophic Lateral Sclerosis by Rowland, Lewis P, Shneider, Neil A

“…Charcot described amyotrophic lateral sclerosis (ALS) in 1874. Despite progress, this creeping paralysis, known colloquially as Lou Gehrig's disease, is still…”
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Monitoring peripheral nerve degeneration in ALS by label-free stimulated Raman scattering imaging by Tian, Feng, Yang, Wenlong, Mordes, Daniel A., Wang, Jin-Yuan, Salameh, Johnny S., Mok, Joanie, Chew, Jeannie, Sharma, Aarti, Leno-Duran, Ester, Suzuki-Uematsu, Satomi, Suzuki, Naoki, Han, Steve S., Lu, Fa-Ke, Ji, Minbiao, Zhang, Rosanna, Liu, Yue, Strominger, Jack, Shneider, Neil A., Petrucelli, Leonard, Xie, X. Sunney, Eggan, Kevin

“…The study of amyotrophic lateral sclerosis (ALS) and potential interventions would be facilitated if motor axon degeneration could be more readily visualized…”
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A Regulatory Circuitry Between Gria2, miR-409, and miR-495 Is Affected by ALS FUS Mutation in ESC-Derived Motor Neurons by Capauto, Davide, Colantoni, Alessio, Lu, Lei, Santini, Tiziana, Peruzzi, Giovanna, Biscarini, Silvia, Morlando, Mariangela, Shneider, Neil A., Caffarelli, Elisa, Laneve, Pietro, Bozzoni, Irene

“…Mutations in fused in sarcoma (FUS) cause amyotrophic lateral sclerosis (ALS). FUS is a multifunctional protein involved in the biogenesis and activity of…”
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A new approach for rare variation collapsing on functional protein domains implicates specific genic regions in ALS by Gelfman, Sahar, Dugger, Sarah, de Araujo Martins Moreno, Cristiane, Ren, Zhong, Wolock, Charles J, Shneider, Neil A, Phatnani, Hemali, Cirulli, Elizabeth T, Lasseigne, Brittany N, Harris, Tim, Maniatis, Tom, Rouleau, Guy A, Brown, Jr, Robert H, Gitler, Aaron D, Myers, Richard M, Petrovski, Slavé, Allen, Andrew, Goldstein, David B, Harms, Matthew B

“…Large-scale sequencing efforts in amyotrophic lateral sclerosis (ALS) have implicated novel genes using gene-based collapsing methods. However, pathogenic…”
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Unexpected similarities between C9ORF72 and sporadic forms of ALS/FTD suggest a common disease mechanism by Conlon, Erin G, Fagegaltier, Delphine, Agius, Phaedra, Davis-Porada, Julia, Gregory, James, Hubbard, Isabel, Kang, Kristy, Kim, Duyang, Phatnani, Hemali, Shneider, Neil A, Manley, James L

“…Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) represent two ends of a disease spectrum with shared clinical, genetic and pathological…”
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Role of primary afferents in the developmental regulation of motor axon synapse numbers on Renshaw cells by Siembab, Valerie C., Gomez-Perez, Laura, Rotterman, Travis M., Shneider, Neil A., Alvarez, Francisco J.

“…ABSTRACT Motor function in mammalian species depends on the maturation of spinal circuits formed by a large variety of interneurons that regulate motoneuron…”
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Characterization of the lncRNA transcriptome in mESC-derived motor neurons: Implications for FUS-ALS by Biscarini, Silvia, Capauto, Davide, Peruzzi, Giovanna, Lu, Lei, Colantoni, Alessio, Santini, Tiziana, Shneider, Neil A., Caffarelli, Elisa, Laneve, Pietro, Bozzoni, Irene

“…Long non-coding RNAs (lncRNAs) are currently recognized as crucial players in nervous system development, function and pathology. In Amyotrophic Lateral…”
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Exome sequencing in amyotrophic lateral sclerosis identifies risk genes and pathways by Cirulli, Elizabeth T., Lasseigne, Brittany N., Petrovski, Slavé, Sapp, Peter C., Dion, Patrick A., Leblond, Claire S., Couthouis, Julien, Lu, Yi-Fan, Wang, Quanli, Krueger, Brian J., Ren, Zhong, Keebler, Jonathan, Han, Yujun, Levy, Shawn E., Boone, Braden E., Wimbish, Jack R., Waite, Lindsay L., Jones, Angela L., Carulli, John P., Day-Williams, Aaron G., Staropoli, John F., Xin, Winnie W., Chesi, Alessandra, Raphael, Alya R., McKenna-Yasek, Diane, Cady, Janet, de Jong, J. M. B. Vianney, Kenna, Kevin P., Smith, Bradley N., Topp, Simon, Miller, Jack, Gkazi, Athina, Al-Chalabi, Ammar, van den Berg, Leonard H., Veldink, Jan, Silani, Vincenzo, Ticozzi, Nicola, Shaw, Christopher E., Baloh, Robert H., Appel, Stanley, Simpson, Ericka, Lagier-Tourenne, Clotilde, Pulst, Stefan M., Gibson, Summer, Trojanowski, John Q., Elman, Lauren, McCluskey, Leo, Grossman, Murray, Shneider, Neil A., Chung, Wendy K., Ravits, John M., Glass, Jonathan D., Sims, Katherine B., Van Deerlin, Vivianna M., Maniatis, Tom, Hayes, Sebastian D., Ordureau, Alban, Swarup, Sharan, Landers, John, Baas, Frank, Allen, Andrew S., Bedlack, Richard S., Harper, J. Wade, Gitler, Aaron D., Rouleau, Guy A., Brown, Robert, Harms, Matthew B., Cooper, Gregory M., Harris, Tim, Myers, Richard M., Goldstein, David B.

“…Amyotrophic lateral sclerosis (ALS) is a devastating neurological disease with no effective treatment. We report the results of a moderate-scale sequencing…”
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Magnetic properties of high-symmetry CuO by Rabinovich, K. S., Samoilenko, L. L., Zhuravleva, A. S., Shneider, A. G.

“…We investigated structural and magnetic properties of CuO-Cu2O heterostructures obtained by high-temperature annealing of copperplate. In samples synthesized…”
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