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

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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Bibliographic Details
Main Authors: Hong Lin, Jordi Magrane, Amy Rattelle, Anna Stepanova, Alexander Galkin, Elisia M. Clark, Yi Na Dong, Sarah M. Halawani, David R. Lynch
Format: Article
Language:English
Published: The Company of Biologists 2017-11-01
Series:Disease Models & Mechanisms
Subjects:
Cerebellum
Friedreich ataxia
Mitochondrial biogenesis
Respiratory chain complex
Neurodegenerative diseases
Online Access:http://dmm.biologists.org/content/10/11/1343

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