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

• Main Authors: Hong Lin (Author), Jordi Magrane (Author), Amy Rattelle (Author), Anna Stepanova (Author), Alexander Galkin (Author), Elisia M. Clark (Author), Yi Na Dong (Author), Sarah M. Halawani (Author), David R. Lynch (Author)
• Format: Book
• Published: The Company of Biologists, 2017-11-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

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;...