Search Results - "Hruska‐Plochan, Marian"

Synaptic FUS accumulation triggers early misregulation of synaptic RNAs in a mouse model of ALS by Sahadevan, Sonu, Hembach, Katharina M., Tantardini, Elena, Pérez-Berlanga, Manuela, Hruska-Plochan, Marian, Megat, Salim, Weber, Julien, Schwarz, Petra, Dupuis, Luc, Robinson, Mark D., De Rossi, Pierre, Polymenidou, Magdalini

“…Mutations disrupting the nuclear localization of the RNA-binding protein FUS characterize a subset of amyotrophic lateral sclerosis patients (ALS-FUS). FUS…”
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Loss of TDP‐43 oligomerization or RNA binding elicits distinct aggregation patterns by Pérez‐Berlanga, Manuela, Wiersma, Vera I, Zbinden, Aurélie, De Vos, Laura, Wagner, Ulrich, Foglieni, Chiara, Mallona, Izaskun, Betz, Katharina M, Cléry, Antoine, Weber, Julien, Guo, Zhongning, Rigort, Ruben, de Rossi, Pierre, Manglunia, Ruchi, Tantardini, Elena, Sahadevan, Sonu, Stach, Oliver, Hruska‐Plochan, Marian, Allain, Frederic H‐T, Paganetti, Paolo, Polymenidou, Magdalini

“…Aggregation of the RNA‐binding protein TAR DNA‐binding protein 43 (TDP‐43) is the key neuropathological feature of neurodegenerative diseases, including…”
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LAG3 is not expressed in human and murine neurons and does not modulate α‐synucleinopathies by Emmenegger, Marc, De Cecco, Elena, Hruska‐Plochan, Marian, Eninger, Timo, Schneider, Matthias M, Barth, Melanie, Tantardini, Elena, de Rossi, Pierre, Bacioglu, Mehtap, Langston, Rebekah G, Kaganovich, Alice, Bengoa‐Vergniory, Nora, Gonzalez‐Guerra, Andrès, Avar, Merve, Heinzer, Daniel, Reimann, Regina, Häsler, Lisa M, Herling, Therese W, Matharu, Naunehal S, Landeck, Natalie, Luk, Kelvin, Melki, Ronald, Kahle, Philipp J, Hornemann, Simone, Knowles, Tuomas P J, Cookson, Mark R, Polymenidou, Magdalini, Jucker, Mathias, Aguzzi, Adriano

“…While the initial pathology of Parkinson’s disease and other α‐synucleinopathies is often confined to circumscribed brain regions, it can spread and…”
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An arrayed genome‐wide perturbation screen identifies the ribonucleoprotein Hnrnpk as rate‐limiting for prion propagation by Avar, Merve, Heinzer, Daniel, Thackray, Alana M, Liu, Yingjun, Hruska‐Plochan, Marian, Sellitto, Stefano, Schaper, Elke, Pease, Daniel P, Yin, Jiang‐An, Lakkaraju, Asvin KK, Emmenegger, Marc, Losa, Marco, Chincisan, Andra, Hornemann, Simone, Polymenidou, Magdalini, Bujdoso, Raymond, Aguzzi, Adriano

“…A defining characteristic of mammalian prions is their capacity for self‐sustained propagation. Theoretical considerations and experimental evidence suggest…”
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TDP-43 extracted from frontotemporal lobar degeneration subject brains displays distinct aggregate assemblies and neurotoxic effects reflecting disease progression rates by Laferrière, Florent, Maniecka, Zuzanna, Pérez-Berlanga, Manuela, Hruska-Plochan, Marian, Gilhespy, Larissa, Hock, Eva-Maria, Wagner, Ulrich, Afroz, Tariq, Boersema, Paul J., Barmettler, Gery, Foti, Sandrine C., Asi, Yasmine T., Isaacs, Adrian M., Al-Amoudi, Ashraf, Lewis, Amanda, Stahlberg, Henning, Ravits, John, De Giorgi, Francesca, Ichas, François, Bezard, Erwan, Picotti, Paola, Lashley, Tammaryn, Polymenidou, Magdalini

“…Accumulation of abnormally phosphorylated TDP-43 (pTDP-43) is the main pathology in affected neurons of people with amyotrophic lateral sclerosis (ALS) and…”
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Macrophage Migration Inhibitory Factor as a Chaperone Inhibiting Accumulation of Misfolded SOD1 by Israelson, Adrian, Ditsworth, Dara, Sun, Shuying, Song, SungWon, Liang, Jason, Hruska-Plochan, Marian, McAlonis-Downes, Melissa, Abu-Hamad, Salah, Zoltsman, Guy, Shani, Tom, Maldonado, Marcus, Bui, Anh, Navarro, Michael, Zhou, Huilin, Marsala, Martin, Kaspar, Brian K., Da Cruz, Sandrine, Cleveland, Don W.

“…Mutations in superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis (ALS), a neurodegenerative disease characterized by loss of motor neurons and…”
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Human neural stem cell replacement therapy for amyotrophic lateral sclerosis by spinal transplantation by Hefferan, Michael P, Galik, Jan, Kakinohana, Osamu, Sekerkova, Gabriela, Santucci, Camila, Marsala, Silvia, Navarro, Roman, Hruska-Plochan, Marian, Johe, Karl, Feldman, Eva, Cleveland, Don W, Marsala, Martin

“…Mutation in the ubiquitously expressed cytoplasmic superoxide dismutase (SOD1) causes an inherited form of Amyotrophic Lateral Sclerosis (ALS). Mutant…”
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Chromatin establishes an immature version of neuronal protocadherin selection during the naive-to-primed conversion of pluripotent stem cells by Almenar-Queralt, Angels, Merkurjev, Daria, Kim, Hong Sook, Navarro, Michael, Ma, Qi, Chaves, Rodrigo S., Allegue, Catarina, Driscoll, Shawn P., Chen, Andrew G., Kohlnhofer, Bridget, Fong, Lauren K., Woodruff, Grace, Mackintosh, Carlos, Bohaciakova, Dasa, Hruska-Plochan, Marian, Tadokoro, Takahiro, Young, Jessica E., El Hajj, Nady, Dittrich, Marcus, Marsala, Martin, Goldstein, Lawrence S. B., Garcia-Bassets, Ivan

“…In the mammalian genome, the clustered protocadherin ( cPCDH ) locus provides a paradigm for stochastic gene expression with the potential to generate a unique…”
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Pig models of neurodegenerative disorders: Utilization in cell replacement-based preclinical safety and efficacy studies by Dolezalova, Dasa, Hruska-Plochan, Marian, Bjarkam, Carsten R., Sørensen, Jens Christian H., Cunningham, Miles, Weingarten, David, Ciacci, Joseph D., Juhas, Stefan, Juhasova, Jana, Motlik, Jan, Hefferan, Michael P., Hazel, Tom, Johe, Karl, Carromeu, Cassiano, Muotri, Alysson, Bui, Jack, Strnadel, Jan, Marsala, Martin

“…ABSTRACT An important component for successful translation of cell replacement‐based therapies into clinical practice is the utilization of large animal models…”
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A scalable solution for isolating human multipotent clinical-grade neural stem cells from ES precursors by Bohaciakova, Dasa, Hruska-Plochan, Marian, Tsunemoto, Rachel, Gifford, Wesley D, Driscoll, Shawn P, Glenn, Thomas D, Wu, Stephanie, Marsala, Silvia, Navarro, Michael, Tadokoro, Takahiro, Juhas, Stefan, Juhasova, Jana, Platoshyn, Oleksandr, Piper, David, Sheckler, Vickie, Ditsworth, Dara, Pfaff, Samuel L, Marsala, Martin

“…A well-characterized method has not yet been established to reproducibly, efficiently, and safely isolate large numbers of clinical-grade multipotent human…”
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Amelioration of motor/sensory dysfunction and spasticity in a rat model of acute lumbar spinal cord injury by human neural stem cell transplantation by van Gorp, Sebastiaan, Leerink, Marjolein, Kakinohana, Osamu, Platoshyn, Oleksandr, Santucci, Camila, Galik, Jan, Joosten, Elbert A, Hruska-Plochan, Marian, Goldberg, Danielle, Marsala, Silvia, Johe, Karl, Ciacci, Joseph D, Marsala, Martin

“…Intraspinal grafting of human neural stem cells represents a promising approach to promote recovery of function after spinal trauma. Such a treatment may serve…”
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Targeted mass spectrometry for monitoring of neural differentiation by Sucha, Rita, Kubickova, Martina, Cervenka, Jakub, Hruska-Plochan, Marian, Bohaciakova, Dasa, Vodickova Kepkova, Katerina, Novakova, Tereza, Budkova, Katerina, Susor, Andrej, Marsala, Martin, Motlik, Jan, Kovarova, Hana, Vodicka, Petr

“…Human multipotent neural stem cells could effectively be used for the treatment of a variety of neurological disorders. However, a defining signature of neural…”
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Effective long-term immunosuppression in rats by subcutaneously implanted sustained-release tacrolimus pellet: Effect on spinally grafted human neural precursor survival by Sevc, Juraj, Goldberg, Danielle, van Gorp, Sebastiaan, Leerink, Marjolein, Juhas, Stefan, Juhasova, Jana, Marsala, Silvia, Hruska-Plochan, Marian, Hefferan, Michael P., Motlik, Jan, Rypacek, Frantisek, Machova, Ludka, Kakinohana, Osamu, Santucci, Camila, Johe, Karl, Lukacova, Nadezda, Yamada, Kazuhiko, Bui, Jack D., Marsala, Martin

“…Achievement of effective, safe and long-term immunosuppression represents one of the challenges in experimental allogeneic and xenogeneic cell and organ…”
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Potent spinal parenchymal AAV9-mediated gene delivery by subpial injection in adult rats and pigs by Miyanohara, Atsushi, Kamizato, Kota, Juhas, Stefan, Juhasova, Jana, Navarro, Michael, Marsala, Silvia, Lukacova, Nada, Hruska-Plochan, Marian, Curtis, Erik, Gabel, Brandon, Ciacci, Joseph, Ahrens, Eric T, Kaspar, Brian K, Cleveland, Don, Marsala, Martin

“…Effective in vivo use of adeno-associated virus (AAV)-based vectors to achieve gene-specific silencing or upregulation in the central nervous system has been…”
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New and emerging roles of small RNAs in neurodegeneration, muscle, cardiovascular and inflammatory diseases by Hruska-Plochan, Marian, Li, Bei, Kyburz, Diego, Krützfeld, Jan, Landmesser, Ulf, Aguzzi, Adriano, Polymenidou, Magdalini

“…Small noncoding RNAs (snRNAs) were discovered more than two decades ago, yet it was not until relatively recently that their important role in genome…”
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The embryonic nucleologenesis during inhibition of major transcriptional activity in bovine preimplantation embryos by Kovalská, Mária, Hruška-Plocháň, Marián, Østrup, Oľga, Adamkov, Marian, Lehotský, Ján, Strejček, František, Statelová, Dagmar, Mikušková, Katarína, Varga, Ivan, Petrovičová, Ida

“…Common features of embryonic genome activation in mammalian and non-mammalian embryos are the colocalization of pre-assembled complexes of maternally inherited…”
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Nuclear Import Receptors Directly Bind to Arginine-Rich Dipeptide Repeat Proteins and Suppress Their Pathological Interactions by Hutten, Saskia, Usluer, Sinem, Bourgeois, Benjamin, Simonetti, Francesca, Odeh, Hana M., Fare, Charlotte M., Czuppa, Mareike, Hruska-Plochan, Marian, Hofweber, Mario, Polymenidou, Magdalini, Shorter, James, Edbauer, Dieter, Madl, Tobias, Dormann, Dorothee

“…Nuclear import receptors, also called importins, mediate nuclear import of proteins and chaperone aggregation-prone cargoes (e.g., neurodegeneration-linked…”
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A model of human neural networks reveals NPTX2 pathology in ALS and FTLD by Hruska-Plochan, Marian, Wiersma, Vera I., Betz, Katharina M., Mallona, Izaskun, Ronchi, Silvia, Maniecka, Zuzanna, Hock, Eva-Maria, Tantardini, Elena, Laferriere, Florent, Sahadevan, Sonu, Hoop, Vanessa, Delvendahl, Igor, Pérez-Berlanga, Manuela, Gatta, Beatrice, Panatta, Martina, van der Bourg, Alexander, Bohaciakova, Dasa, Sharma, Puneet, De Vos, Laura, Frontzek, Karl, Aguzzi, Adriano, Lashley, Tammaryn, Robinson, Mark D., Karayannis, Theofanis, Mueller, Martin, Hierlemann, Andreas, Polymenidou, Magdalini

“…Human cellular models of neurodegeneration require reproducibility and longevity, which is necessary for simulating age-dependent diseases. Such systems are…”
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Hypertonic Stress Causes Cytoplasmic Translocation of Neuronal, but Not Astrocytic, FUS due to Impaired Transportin Function by Hock, Eva-Maria, Maniecka, Zuzanna, Hruska-Plochan, Marian, Reber, Stefan, Laferrière, Florent, Sahadevan M.K., Sonu, Ederle, Helena, Gittings, Lauren, Pelkmans, Lucas, Dupuis, Luc, Lashley, Tammaryn, Ruepp, Marc-David, Dormann, Dorothee, Polymenidou, Magdalini

“…The primarily nuclear RNA-binding protein FUS (fused in sarcoma) forms pathological cytoplasmic inclusions in a subset of early-onset amyotrophic lateral…”
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Lentiviral-Mediated Transgenesis in Songbirds by Liu, Wan-Chun, Hruska-Plochan, Marian, Miyanohara, Atsushi

“…Transgenesis involves the insertion of an exogenous gene into an animal's genome, which allows the identification of the expressed phenotypes in brain function…”
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