The MCK mouse heart model of Friedreich's ataxia: Alterations in iron-regulated proteins and cardiac hypertrophy are limited by iron chelation

There is no effective treatment for the cardiomyopathy of the most common autosomal recessive ataxia, Friedreich's ataxia (FA). The identification of potentially toxic mitochondrial (MIT) iron (Fe) deposits in FA suggests that Fe plays a role in its pathogenesis. This study used the muscle crea...

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Autori principali: Whitnall, Megan, Rahmanto, Yohan Suryo, Sutak, Robert, Xu, Xiangcong, Becker, Erika M., Mikhael, Marc R., Ponka, Prem, Richardson, Des R.
Natura: Artigo
Lingua:Inglês
Pubblicazione: National Academy of Sciences 2008
Soggetti:
Biological Sciences
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC2474513/
https://ncbi.nlm.nih.gov/pubmed/18621680
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.0804261105
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