Zobrazeno 1 - 10
of 12
pro vyhledávání: '"Nicolas Delestrée"'
Autor:
Giulietta M. Riboldi, Irene Faravelli, Takaaki Kuwajima, Nicolas Delestrée, Georgia Dermentzaki, Mariangels De Planell-Saguer, Paola Rinchetti, Le Thi Hao, Christine C. Beattie, Stefania Corti, Serge Przedborski, George Z. Mentis, Francesco Lotti
Publikováno v:
Nature Communications, Vol 12, Iss 1, Pp 1-18 (2021)
Sumoylation is important for the assembly and function of the SMN complex, which plays a central role in RNA processing. Here the authors show that loss of this posttranslational modification impairs the ability of SMN to correct selective deficits i
Externí odkaz:
https://doaj.org/article/9ae40bf770e34a49910b6f8f9467d2e2
Autor:
Aleksandra Vukojicic, Nicolas Delestrée, Emily V. Fletcher, John G. Pagiazitis, Sethu Sankaranarayanan, Ted A. Yednock, Ben A. Barres, George Z. Mentis
Publikováno v:
Cell Reports, Vol 29, Iss 10, Pp 3087-3100.e7 (2019)
Summary: Movement is an essential behavior requiring the assembly and refinement of spinal motor circuits. However, the mechanisms responsible for circuit refinement and synapse maintenance are poorly understood. Similarly, the molecular mechanisms b
Externí odkaz:
https://doaj.org/article/b00b1ef1c3644734996365a67dfccaaa
Publikováno v:
eLife, Vol 7 (2018)
In amyotrophic lateral sclerosis (ALS) and animal models of ALS, including SOD1-G93A mice, disassembly of the neuromuscular synapse precedes motor neuron loss and is sufficient to cause a decline in motor function that culminates in lethal respirator
Externí odkaz:
https://doaj.org/article/d3615645dc99482ea7b9f2d156a2a4c8
Autor:
Serge Przedborski, Takaaki Kuwajima, Mariàngels de Planell-Saguer, Georgia Dermentzaki, Le Thi Hao, Giulietta Riboldi, Francesco Lotti, George Z. Mentis, Christine C. Beattie, Nicolas Delestrée, Stefania Corti, Irene Faravelli, Paola Rinchetti
Publikováno v:
Nature Communications, Vol 12, Iss 1, Pp 1-18 (2021)
Nature Communications
Nature Communications
SMN is a ubiquitously expressed protein and is essential for life. SMN deficiency causes the neurodegenerative disease spinal muscular atrophy (SMA), the leading genetic cause of infant mortality. SMN interacts with itself and other proteins to form
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8064d5c988f9ead8c81b5560c6706145
https://doaj.org/article/9ae40bf770e34a49910b6f8f9467d2e2
https://doaj.org/article/9ae40bf770e34a49910b6f8f9467d2e2
Autor:
Alexander Stephan, Lingling Kong, Thomas O. Crawford, Nikolai Naryshkin, Christian M. Simon, Nicolas Delestrée, Crystal Davis, Melissa E. Crowder, Xixi Xu, George Z. Mentis, Zachary A. King, Marla Weetall, Cera W. Hassinan, Chloe C. Grzyb, Marco Petrillo, Kathryn J. Swoboda, Cathleen M. Lutz, Celeste M. Pilato, Charlotte J. Sumner, Xin Zhao, David Valdivia, Daniel M. Ramos, Jae Hong Park
Publikováno v:
Sci Transl Med
Gene replacement and pre-mRNA splicing modifier therapies represent breakthrough gene targeting treatments for the neuromuscular disease spinal muscular atrophy (SMA), but mechanisms underlying variable efficacy of treatment are incompletely understo
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c0801efb8fc06a120d7f26d49d1f1401
https://doi.org/10.1126/scitranslmed.abb6871
https://doi.org/10.1126/scitranslmed.abb6871
Autor:
Ivan Tattoli, Meaghan Van Alstyne, Lamya S. Shihabuddin, Chaolin Zhang, Nicolas Delestrée, Yocelyn Recinos, Livio Pellizzoni, George Z. Mentis, Eileen Workman
Publikováno v:
Nature neuroscience. 24(7)
The neurodegenerative disease spinal muscular atrophy (SMA) is caused by deficiency in the survival motor neuron (SMN) protein. Currently approved SMA treatments aim to restore SMN, but the potential for SMN expression beyond physiological levels is
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::dd00f6bc6af2d891a8ed825422c78924
https://pubmed.ncbi.nlm.nih.gov/35656408
https://pubmed.ncbi.nlm.nih.gov/35656408
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
Autor:
Jennifer K. Lee, Peter T. Hallock, Steven J. Burden, Katherine D. Gribble, Nicolas Delestrée, George Z. Mentis, Natalie Kim, Michael Granato, Robert C. Froemke, Leonor Remédio
Publikováno v:
Genes & Development. 30:1058-1069
Motor axons approach muscles that are prepatterned in the prospective synaptic region. In mice, prepatterning of acetylcholine receptors requires Lrp4, a LDLR family member, and MuSK, a receptor tyrosine kinase. Lrp4 can bind and stimulate MuSK, stro
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d21c99235d57b586d2ff3ab9d9418ae5
https://doi.org/10.1101/gad.279745.116
https://doi.org/10.1101/gad.279745.116
Autor:
Charles J. Heckman, Sherif M. Elbasiouny, Daniel Zytnicki, Caroline Iglesias, Marin Manuel, Nicolas Delestrée
Publikováno v:
The Journal of Physiology. 592:1687-1703
In amyotrophic lateral sclerosis (ALS), an adult onset disease in which there is progressive degeneration of motoneurones, it has been suggested that an intrinsic hyperexcitability of motoneurones (i.e. an increase in their firing rates), contributes
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::5d3860d36d07d851b1bca7efcde53944
https://doi.org/10.1113/jphysiol.2013.265843
https://doi.org/10.1113/jphysiol.2013.265843