Extra-mitochondrial mouse frataxin and its implications for mouse models of Friedreich’s ataxia

Mature frataxin is essential for the assembly of iron–sulfur cluster proteins including a number of mitochondrial enzymes. Reduced levels of mature frataxin (81-20) in human subjects caused by the genetic disease Friedreich’s ataxia results in decreased mitochondrial function, neurodegeneration, and...

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Detalles Bibliográficos
Autores principales: Weng, Liwei, Laboureur, Laurent, Wang, Qingqing, Guo, Lili, Xu, Peining, Gottlieb, Leah, Lynch, David R., Mesaros, Clementina, Blair, Ian A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2020
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7519113/
https://www.ncbi.nlm.nih.gov/pubmed/32978498
http://dx.doi.org/10.1038/s41598-020-72884-w

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7519113/
https://www.ncbi.nlm.nih.gov/pubmed/32978498
http://dx.doi.org/10.1038/s41598-020-72884-w

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