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A role for astrocytes in cerebellar deficits in frataxin deficiency: Protection by insulin-like growth factor I

Franco, C. ; Genis, L. ; Navarro, J. A. ; Perez-Domper, P. ; Fernandez, A. M. ; Schneuwly, S. ; Torres Alemán, I.



Zusammenfassung

Inherited neurodegenerative diseases such as Friedreich's ataxia (FRDA), produced by deficiency of the mitochondrial chaperone frataxin (Fxn), shows specific neurological deficits involving different subset of neurons even though deficiency of Fxn is ubiquitous. Because astrocytes are involved in neurodegeneration, we analyzed whether they are also affected by frataxin deficiency and contribute ...

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