Arginine butyrate per os protects mdx mice against cardiomyopathy, kyphosis and changes in axonal excitability

Autor: Sara Vianello, Sophie Bouyon, Evelyne Benoit, Catherine Sebrié, Delphine Boerio, Marc Herbin, Morgane Roulot, Yves Fromes, Sabine de la Porte

Publikováno v: Neurobiology of Disease, Vol 71, Iss , Pp 325-333 (2014)

Duchenne muscular dystrophy (DMD) is a progressive neuromuscular disease caused by lack of dystrophin, a sub-sarcolemmal protein, which leads to dramatic muscle deterioration. We studied in mdx mice, the effects of oral administration of arginine but

Externí odkaz: https://doaj.org/article/33657da37cff4ec583b976a8f26d6b70

A mouse model of schwartz-jampel syndrome reveals myelinating schwann cell dysfunction with persistent axonal depolarization in vitro and distal peripheral nerve hyperexcitability when perlecan is lacking

Autor: Maria Laura Feltri, Jérôme Devaux, Frédérique René, Claire-Sophie Davoine, Sophie Nicole, Andoni Echaniz-Laguna, Marie Bangratz, Desirée Zambroni, Delphine Boerio, Nadège Sarrazin, Bertrand Fontaine, Evelyne Benoit

Publikováno v: American Journal of Pathology
American Journal of Pathology, 2012, 180 (5), pp.2040-55. ⟨10.1016/j.ajpath.2012.01.035⟩
American Journal of Pathology, American Society for Investigative Pathology, 2012, 180 (5), pp.2040-55. ⟨10.1016/j.ajpath.2012.01.035⟩

International audience; Congenital peripheral nerve hyperexcitability (PNH) is usually associated with impaired function of voltage-gated K(+) channels (VGKCs) in neuromyotonia and demyelination in peripheral neuropathies. Schwartz-Jampel syndrome (S

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::1595fef080479b7ab9c0fda869efe6b6
https://hal.science/hal-00701186