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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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Bibliografiske detaljer
Udgivet i:Dis Model Mech
Main Authors: Lin, Hong, Magrane, Jordi, Rattelle, Amy, Stepanova, Anna, Galkin, Alexander, Clark, Elisia M., Dong, Yi Na, Halawani, Sarah M., Lynch, David R.
Format: Artigo
Sprog:Inglês
Udgivet: The Company of Biologists Ltd 2017
Fag:
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC5719255/
https://ncbi.nlm.nih.gov/pubmed/29125827
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1242/dmm.030502
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