HDAC6 inhibition reverses axonal transport defects in motor neurons derived from FUS-ALS patients

Autor: Wenting Guo, Maximilian Naujock, Laura Fumagalli, Tijs Vandoorne, Pieter Baatsen, Ruben Boon, Laura Ordovás, Abdulsamie Patel, Marc Welters, Thomas Vanwelden, Natasja Geens, Tine Tricot, Veronick Benoy, Jolien Steyaert, Cynthia Lefebvre-Omar, Werend Boesmans, Matthew Jarpe, Jared Sterneckert, Florian Wegner, Susanne Petri, Delphine Bohl, Pieter Vanden Berghe, Wim Robberecht, Philip Van Damme, Catherine Verfaillie, Ludo Van Den Bosch

Publikováno v: Nature Communications, Vol 8, Iss 1, Pp 1-15 (2017)

Amyotrophic lateral sclerosis (ALS) leads to selective loss of motor neurons. Using motor neurons derived from induced pluripotent stem cells from patients with ALS and FUS mutations, the authors demonstrate that axonal transport deficits that are ob

Externí odkaz: https://doaj.org/article/f59dc3e6db71437985afab751df3c753

HDAC6 inhibition reverses axonal transport defects in motor neurons derived from FUS-ALS patients

Autor: Delphine Bohl, Natasja Geens, Wenting Guo, Tijs Vandoorne, Cynthia Lefebvre-Omar, Catherine M. Verfaillie, Ludo Van Den Bosch, Thomas Vanwelden, Wim Robberecht, Laura Fumagalli, Werend Boesmans, Laura Ordovás, Jared Sterneckert, Jolien Steyaert, Tine Tricot, Maximilian Naujock, Ruben Boon, Susanne Petri, Marc Welters, Pieter Vanden Berghe, Philip Van Damme, Florian Wegner, Abdulsamie Patel, Veronick Benoy, Matthew Jarpe, Pieter Baatsen

Publikováno v: Nature Communications, Vol 8, Iss 1, Pp 1-15 (2017)
Nature Communications
Nature Communications, 2017, 8 (1), pp.394-405. ⟨10.1038/s41467-017-00911-y⟩
Zaguán. Repositorio Digital de la Universidad de Zaragoza
instname
Nature Communications, Nature Publishing Group, 2017, 8 (1), pp.394-405. ⟨10.1038/s41467-017-00911-y⟩
Cell Stress

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder due to selective loss of motor neurons (MNs). Mutations in the fused in sarcoma (FUS) gene can cause both juvenile and late onset ALS. We generated and characteri

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::1007a9e7438d2ee615cd9ac4be4a3552
http://link.springer.com/article/10.1038/s41467-017-00911-y

Mitochondrial defect in muscle precedes neuromuscular junction degeneration and motor neuron death in CHCHD10S59L/+ mouse

Autor: Blandine Madji Hounoum, Françoise Lespinasse, Alessandra Mauri-Crouzet, Delphine Bohl, Fanny Mochel, Emmanuelle Génin, Jean-Ehrland Ricci, Aude Chiot, Véronique Paquis-Flucklinger, Charlotte Cochaud, Julien Neveu, Cynthia Lefebvre-Omar, Sandra Lacas-Gervais, Konstantina Fragaki, Stéphanie Bigou, Gaëlle Augé, Christian S. Lobsiger, Séverine Boillée, Sylvie Bannwarth, Baptiste Ropert

Publikováno v: Acta Neuropathologica
Acta Neuropathologica, Springer Verlag, 2019, 138 (1), pp.123-145. ⟨10.1007/s00401-019-01988-z⟩

Recently, we provided genetic basis showing that mitochondrial dysfunction can trigger motor neuron degeneration, through identification of CHCHD10 encoding a mitochondrial protein. We reported patients, carrying the p.Ser59Leu heterozygous mutation

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e531dfbddb5cd0ba97219486d7be7d83
https://hal.archives-ouvertes.fr/hal-03014387