Zobrazeno 1 - 10
of 32
pro vyhledávání: '"pathology [Motor Neurons]"'
Autor:
Pérez-Millan, Agnès, Borrego-Écija, Sergi, van Swieten, John C., Jiskoot, Lize, Moreno, Fermin, Laforce, Robert, Graff, Caroline, Masellis, Mario, Tartaglia, Maria Carmela, Rowe, James B., Borroni, Barbara, Finger, Elizabeth, Synofzik, Matthis, Galimberti, Daniela, Vandenberghe, Rik, de Mendonça, Alexandre, Butler, Chris R., Gerhard, Alexander, Ducharme, Simon, Le Ber, Isabelle, Santana, Isabel, Pasquier, Florence, Levin, Johannes, Otto, Markus, Sorbi, Sandro, Tiraboschi, Pietro, Seelaar, Harro, Langheinrich, Tobias, Rohrer, Jonathan D., Sala-Llonch, Roser, Sánchez-Valle, Raquel, GENFI The Genetic FTD Initiative, Ullgren, Abbe, Rollin, Adeline, Camuzat, Agnès, Esteve, Aitana Sogorb, Gabilondo, Alazne, Lladó, Albert, Benussi, Alberto, Brice, Alexis, Gorostidi, Ana, Verdelho, Ana, Arighi, Andrea, Antonell, Anna, Bertrand, Anne, Engel, Annerose, Vogels, Annick, Bouzigues, Arabella, Funkiewiez, Aurélie, Nacmias, Benedetta, Bender, Benjamin, Ferrari, Camilla, Wilke, Carlo, Heller, Carolin, Maruta, Carolina, Greaves, Caroline V., Timberlake, Carolyn, Ferreira, Catarina B., Prix, Catharina, Fenoglio, Chiara, Shoesmith, Christen, Polito, Cristina, Rinaldi, Daisy, Saracino, Dario, Cash, David, Thomas, David L., Tang-Wai, David, Duro, Diana, Rogaeva, Ekaterina, Scarpini, Elio, Wlasich, Elisabeth, Buratti, Emanuele, Todd, Emily, Premi, Enrico, do Couto, Frederico Simões, Miltenberger, Gabriel, Lombardi, Gemma, Rossi, Giacomina, Fumagalli, Giorgio, Giaccone, Giorgio, Di Fede, Giuseppe, Kuchcinski, Gregory, Benotmane, Hanya, Zetterberg, Henrik, Swift, Imogen J., Poos, Jackie, M. Papma, Janne, Nicholas, Jennifer, Durães, João, Lombardi, Jolina, Juncà-Parella, Jordi, Sarto, Jordi, Villanua, Jorge, Samra, Kiran, Poesen, Koen, Öijerstedt, Linn, Graf, Lisa, Giannini, Lucia, Russell, Lucy L., Leitão, Maria João, Almeida, Maria Rosario, Serpente, Maria, Lima, Marisa, Cañada, Marta, Bocchetta, Martina, Polyakova, Maryna, Vandenbulcke, Mathieu, Bertoux, Maxime, Veldsman, Michele, Castelo-Branco, Miguel, Tábuas-Pereira, Miguel, Tainta, Mikel, Balasa, Mircea, Zulaica, Miren, Freedman, Morris, Barandiaran, Myriam, Bargalló, Nuria, Wagemann, Olivia, Colliot, Olivier, Caroppo, Paola, Alves, Patricia, Thompson, Paul, Rosa-Neto, Pedro, Van Damme, Philip, Shafei, Rachelle, Convery, Rhian S., van Minkelen, Rick, Bartha, Robart, Gasparotti, Roberto, Keren, Ron, Rademakers, Rosa, Bruffaerts, Rose, Sayah, Sabrina, Black, Sandra, Loosli, Sandra, Mitchell, Sara, Prioni, Sara, Anderl-Straub, Sarah, Gauthier, Serge, Afonso, Sónia, Schönecker, Sonja, Gazzina, Stefano, Lebouvier, Thibaud, Cope, Thomas, Rittman, Timothy, Hoegen, Tobias, Bessi, Valentina, Cantoni, Valentina, Redaelli, Veronica, Jelic, Vesna, Deramecourt, Vincent, Borracci, Vittoria
Publikováno v:
Journal of neurology
Journal of neurology 270(3), 1573-1586 (2022). doi:10.1007/s00415-022-11435-x
Journal of neurology 270(3), 1573-1586 (2022). doi:10.1007/s00415-022-11435-x
Background and objectives The C9orf72 expansion is the most common genetic cause of frontotemporal dementia (FTD) and/or motor neuron disease (MND). Corticospinal degeneration has been described in post-mortem neuropathological studies in these patie
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::06b2afc542f6412fadfd24dab268155c
https://doi.org/10.1007/s00415-022-11435-x
https://doi.org/10.1007/s00415-022-11435-x
Autor:
Florian Wegner, Selma Staege, Andreas Hermann, Stefan Stefanov, Hannes Glass, Susanne Petri, Dieter Edbauer, Rajat Bhatnagar, Antje Janosch, Petra Freitag, Anne-Karin Kahlert, Marc Bickle, Jared Sterneckert, Tanja Richter, Arun Pal, Carina Schludi, Walter Just, Norman Kalmbach, Masin Abo-Rady
Publikováno v:
Stem Cell Reports, Vol 14, Iss 3, Pp 390-405 (2020)
Stem Cell Reports
Stem cell reports 14(3), 390-405 (2020). doi:10.1016/j.stemcr.2020.01.010
Stem Cell Reports
Stem cell reports 14(3), 390-405 (2020). doi:10.1016/j.stemcr.2020.01.010
Summary In amyotrophic lateral sclerosis (ALS) motor neurons (MNs) undergo dying-back, where the distal axon degenerates before the soma. The hexanucleotide repeat expansion (HRE) in C9ORF72 is the most common genetic cause of ALS, but the mechanism
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3e36d802ed14d96d26a9814e300c2698
http://www.sciencedirect.com/science/article/pii/S221367112030031X
http://www.sciencedirect.com/science/article/pii/S221367112030031X
Autor:
Evelien Van Schoor, Simona Ospitalieri, Sebastiaan Moonen, Sandra O. Tomé, Alicja Ronisz, Orkun Ok, Jochen Weishaupt, Albert C. Ludolph, Philip Van Damme, Ludo Van Den Bosch, Dietmar Rudolf Thal
Publikováno v:
Acta neuropathologica 144(3), 393-411 (2022). doi:10.1007/s00401-022-02466-9
Amyotrophic lateral sclerosis (ALS) is characterized by the degeneration of motor neurons in the motor cortex, brainstem, and spinal cord. Although ALS is considered a motor neuron disorder, neuroinflammation also plays an important role. Recent evid
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ec3c53395a7b4e2afdc364d08dc754e8
https://pubmed.ncbi.nlm.nih.gov/35867112
https://pubmed.ncbi.nlm.nih.gov/35867112
Autor:
Stefanie Schreiber, Jose Bernal, Philipp Arndt, Frank Schreiber, Patrick Müller, Lorena Morton, Rüdiger Christian Braun-Dullaeus, Maria Del Carmen Valdés-Hernández, Roberto Duarte, Joanna Marguerite Wardlaw, Sven Günther Meuth, Grazia Mietzner, Stefan Vielhaber, Ildiko Rita Dunay, Alexander Dityatev, Solveig Jandke, Hendrik Mattern
Publikováno v:
Schreiber, S, Bernal, J, Arndt, P, Schreiber, F, Müller, P, Morton, L, Braun-Dullaeus, R C, Valdés-Hernández, M D C, Duarte, R, Wardlaw, J M, Meuth, S G, Mietzner, G, Vielhaber, S, Dunay, I R, Dityatev, A, Jandke, S & Mattern, H 2023, ' Brain Vascular Health in ALS Is Mediated through Motor Cortex Microvascular Integrity ', Cells, vol. 12, no. 6 . https://doi.org/10.3390/cells12060957
Cells 12(6), 957 (2023). doi:10.3390/cells12060957 special issue: "Amyotrophic Lateral Sclerosis: Understanding the Pathogenetic Mechanisms for the Development of New Therapies"
Cells 12(6), 957 (2023). doi:10.3390/cells12060957 special issue: "Amyotrophic Lateral Sclerosis: Understanding the Pathogenetic Mechanisms for the Development of New Therapies"
Brain vascular health appears to be critical for preventing the development of amyotrophic lateral sclerosis (ALS) and slowing its progression. ALS patients often demonstrate cardiovascular risk factors and commonly suffer from cerebrovascular diseas
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::38970d61fb7a93845bd191b92d860f9f
https://doi.org/10.3390/cells12060957
https://doi.org/10.3390/cells12060957
Publikováno v:
International Journal of Molecular Sciences, Vol 22, Iss 8042, p 8042 (2021)
International Journal of Molecular Sciences
Volume 22
Issue 15
International journal of molecular sciences 22(15), 8042 (2021). doi:10.3390/ijms22158042 special issue: "The Mechanisms Regulation of the Neuromuscular Dysfunctions"
International Journal of Molecular Sciences
Volume 22
Issue 15
International journal of molecular sciences 22(15), 8042 (2021). doi:10.3390/ijms22158042 special issue: "The Mechanisms Regulation of the Neuromuscular Dysfunctions"
Amyotrophic lateral sclerosis (ALS) is a progressive disease leading to the degeneration of motor neurons (MNs). Neuroinflammation is involved in the pathogenesis of ALS
however, interactions of specific immune cell types and MNs are not well st
however, interactions of specific immune cell types and MNs are not well st
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=pmid_dedup__::e97a16541ebba52bbb0737e22c38e0a4
https://www.mdpi.com/1422-0067/22/15/8042
https://www.mdpi.com/1422-0067/22/15/8042
Autor:
Daniel Zytnicki, Marcin Bączyk, Nicolas Doisne, Clémence Martinot, Najwa Ouali Alami, Barbara Commisso, Nicolas Delestrée, Francesco Roselli, David Bayer, Marin Manuel, Linyun Tang, Wayne N. Frankel
Publikováno v:
Journal of Experimental Medicine
Journal of Experimental Medicine, Rockefeller University Press, 2020, 217 (8), ⟨10.1084/jem.20191734⟩
The Journal of Experimental Medicine
Journal of experimental medicine 217(8), e20191734 (2020). doi:10.1084/jem.20191734
Journal of Experimental Medicine, Rockefeller University Press, 2020, 217 (8), ⟨10.1084/jem.20191734⟩
The Journal of Experimental Medicine
Journal of experimental medicine 217(8), e20191734 (2020). doi:10.1084/jem.20191734
This study demonstrates that, in a mouse model of ALS, excitatory synapses on spinal motoneurons are disrupted. Activation of the cAMP/PKA pathway reverts the functional and structural synaptic deficits and ameliorates disease markers by increasing m
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a1ee2bfc13e2b37563ad14e712f1530a
https://doi.org/10.1084/jem.20191734
https://doi.org/10.1084/jem.20191734
Publikováno v:
International Journal of Molecular Sciences, Vol 21, Iss 3564, p 3564 (2020)
International journal of molecular sciences 21(10), 3564-(2020). doi:10.3390/ijms21103564
International Journal of Molecular Sciences
Volume 21
Issue 10
International journal of molecular sciences 21(10), 3564-(2020). doi:10.3390/ijms21103564
International Journal of Molecular Sciences
Volume 21
Issue 10
Amyotrophic lateral sclerosis (ALS) is the most common and devastating motor neuron (MN) disease. Its pathophysiological cascade is still enigmatic. More than 90% of ALS patients suffer from sporadic ALS, which makes it specifically demanding to gene
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4899d494be25a4cf5b736645c5fa4e24
https://www.mdpi.com/1422-0067/21/10/3564
https://www.mdpi.com/1422-0067/21/10/3564
Autor:
Ian Casci, Angelo Poletti, Julia Japtok, Claudia Moebius, Karl Hackmann, Marc Bickle, Udai Bhan Pandey, Barbara Klink, Ellen Koerner, Lydia Reinhardt, Antje Janosch, Anthony A. Hyman, Andreas Hermann, Simon Alberti, Lara Marrone, Cordula Andree, Jared Sterneckert, Peter Reinhardt, Ina Poser, Hyun O. Lee, Maria Elena Cicardi
Publikováno v:
Stem Cell Reports
Stem Cell Reports, Vol 10, Iss 2, Pp 375-389 (2018)
Stem cell reports 10(2), 375-389 (2018). doi:10.1016/j.stemcr.2017.12.018
Stem Cell Reports, Vol 10, Iss 2, Pp 375-389 (2018)
Stem cell reports 10(2), 375-389 (2018). doi:10.1016/j.stemcr.2017.12.018
Summary Perturbations in stress granule (SG) dynamics may be at the core of amyotrophic lateral sclerosis (ALS). Since SGs are membraneless compartments, modeling their dynamics in human motor neurons has been challenging, thus hindering the identifi
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::367a59d6fc57ca1ec0aeef1d84ed53ba
https://doi.org/10.1016/j.stemcr.2017.12.018
https://doi.org/10.1016/j.stemcr.2017.12.018
Autor:
Chantal Sellier, Nick H.M. van Bakel, Amila Zuko, Céline Sijlmans, Sina Mersmann, Anne-Laurence Boutillier, Annemarie Hübers, Amr Aly, Marina Wagner, Stéphane Dieterlé, Moushami Mallik, Marc-Antoine Goy, Clotilde Lagier-Tourenne, Gina Picchiarelli, Albert C. Ludolph, Luc Dupuis, Erik Storkebaum, Julia Higelin, Angela Rosenbohm, Li Zhang, Tobias M. Boeckers, Marije Been, Maria Demestre, Jelena Scekic-Zahirovic, Nadia Messaddeq, Inmaculada Sanjuan-Ruiz
Publikováno v:
Nature Neuroscience
Nature Neuroscience, Nature Publishing Group, 2019, 22 (11), pp.1793-1805. ⟨10.1038/s41593-019-0498-9⟩
Nature neuroscience
Nature Neuroscience, 22, 1793-1805
Nature reviews / Neuroscience 22(11), 1793-1805 (2019). doi:10.1038/s41593-019-0498-9
Nature Neuroscience, 22, 11, pp. 1793-1805
Nature Neuroscience, Nature Publishing Group, 2019, 22 (11), pp.1793-1805. ⟨10.1038/s41593-019-0498-9⟩
Nature neuroscience
Nature Neuroscience, 22, 1793-1805
Nature reviews / Neuroscience 22(11), 1793-1805 (2019). doi:10.1038/s41593-019-0498-9
Nature Neuroscience, 22, 11, pp. 1793-1805
Neuromuscular junction (NMJ) disruption is an early pathogenic event in amyotrophic lateral sclerosis (ALS). Yet, direct links between NMJ pathways and ALS-associated genes such as FUS, whose heterozygous mutations cause aggressive forms of ALS, rema
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a29ee2725324cd47aad72a1d21ded34f
https://hal.archives-ouvertes.fr/hal-02406369
https://hal.archives-ouvertes.fr/hal-02406369
Publikováno v:
Singapore : Springer Singapore, Advances in Experimental Medicine and Biology 1175, 227-272 (2019). doi:10.1007/978-981-13-9913-8_10
Neuroglia in Neurodegenerative Diseases / Verkhratsky, Alexei (Editor) ; Singapore : Springer Singapore, 2019, Chapter 10 ; ISSN: 0065-2598=2214-8019 ; ISBN: 978-981-13-9912-1=978-981-13-9913-8 ; doi:10.1007/978-981-13-9913-8
Neuroglia in Neurodegenerative Diseases ISBN: 9789811399121
Neuroglia in Neurodegenerative Diseases / Verkhratsky, Alexei (Editor) ; Singapore : Springer Singapore, 2019, Chapter 10 ; ISSN: 0065-2598=2214-8019 ; ISBN: 978-981-13-9912-1=978-981-13-9913-8 ; doi:10.1007/978-981-13-9913-8
Neuroglia in Neurodegenerative Diseases ISBN: 9789811399121
Motor neuron disorders are highly debilitating and mostly fatal conditions for which only limited therapeutic options are available. To overcome this limitation and develop more effective therapeutic strategies, it is critical to discover the pathoge
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::aa90e8108c7b91a8e556332666d1a7a5