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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Wedi'i Gadw mewn:
Manylion Llyfryddiaeth
Cyhoeddwyd yn:FASEB J
Prif Awduron: 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
Fformat: Artigo
Iaith:Inglês
Cyhoeddwyd: Federation of American Societies for Experimental Biology 2018
Pynciau:
Research
Mynediad Ar-lein:https://ncbi.nlm.nih.gov/pmc/articles/PMC5888399/
https://ncbi.nlm.nih.gov/pubmed/29097503
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1096/fj.201700182RRR
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