Ryanodine channel complex stabilizer compound S48168/ARM210 as a disease modifier in dystrophin-deficient mdx mice: proof-of-concept study and independent validation of efficacy

Muscle fibers lacking dystrophin undergo a long-term alteration of Ca(2+) homeostasis, partially caused by a leaky Ca(2+) release ryanodine (RyR) channel. S48168/ARM210, an RyR calcium release channel stabilizer (a Rycal compound), is expected to enhance the rebinding of calstabin to the RyR channel...

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Detalles Bibliográficos
Autores principales: Capogrosso, Roberta Francesca, Mantuano, Paola, Uaesoontrachoon, Kitipong, Cozzoli, Anna, Giustino, Arcangela, Dow, Todd, Srinivassane, Sadish, Filipovic, Marina, Bell, Christina, Vandermeulen, Jack, Massari, Ada Maria, De Bellis, Michela, Conte, Elena, Pierno, Sabata, Camerino, Giulia Maria, Liantonio, Antonella, Nagaraju, Kanneboyina, De Luca, Annamaria
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Federation of American Societies for Experimental Biology 2018
Materias:
Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5888399/
https://www.ncbi.nlm.nih.gov/pubmed/29097503
http://dx.doi.org/10.1096/fj.201700182RRR

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