Early cerebellar deficits in mitochondrial biogenesis and respiratory chain complexes in the KIKO mouse model of Friedreich ataxia

Friedreich ataxia (FRDA), the most common recessive inherited ataxia, results from deficiency of frataxin, a small mitochondrial protein crucial for iron-sulphur cluster formation and ATP production. Frataxin deficiency is associated with mitochondrial dysfunction in FRDA patients and animal models;...

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Detalles Bibliográficos
Autores principales: Lin, Hong, Magrane, Jordi, Rattelle, Amy, Stepanova, Anna, Galkin, Alexander, Clark, Elisia M., Dong, Yi Na, Halawani, Sarah M., Lynch, David R.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Company of Biologists Ltd 2017
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5719255/
https://www.ncbi.nlm.nih.gov/pubmed/29125827
http://dx.doi.org/10.1242/dmm.030502

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