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The smoothened agonist SAG reduces mitochondrial dysfunction and neurotoxicity of frataxin-deficient astrocytes

Abstract Background Friedreich’s ataxia is a rare hereditary neurodegenerative disease caused by decreased levels of the mitochondrial protein frataxin. Similar to other neurodegenerative pathologies, previous studies suggested that astrocytes might contribute to the progression of the disease. To f...

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Main Authors: Andrés Vicente-Acosta, Alfredo Giménez-Cassina, Javier Díaz-Nido, Frida Loria
Format: Artigo
Language:Inglês
Published: BMC 2022-04-01
Series:Journal of Neuroinflammation
Subjects:
Frataxin
Reactive astrocytes
Sonic hedgehog
Smoothened agonist
Mitochondrial dysfunction
Neurotoxicity
Online Access:https://doi.org/10.1186/s12974-022-02442-w
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