Therapeutic effects of the mitochondrial ROS-redox modulator KH176 in a mammalian model of Leigh Disease

Leigh Disease is a progressive neurometabolic disorder for which a clinical effective treatment is currently still lacking. Here, we report on the therapeutic efficacy of KH176, a new chemical entity derivative of Trolox, in Ndufs4 (−/−) mice, a mammalian model for Leigh Disease. Using in vivo brain...

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Pubblicato in:Sci Rep
Autori principali: de Haas, Ria, Das, Devashish, Garanto, Alejandro, Renkema, Herma G., Greupink, Rick, van den Broek, Petra, Pertijs, Jeanne, Collin, Rob W. J., Willems, Peter, Beyrath, Julien, Heerschap, Arend, Russel, Frans G., Smeitink, Jan A.
Natura: Artigo
Lingua:Inglês
Pubblicazione: Nature Publishing Group UK 2017
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Article
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC5601915/
https://ncbi.nlm.nih.gov/pubmed/28916769
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/s41598-017-09417-5
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