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A new FRDA mouse model [Fxn(null):YG8s(GAA) > 800] with more than 800 GAA repeats

INTRODUCTION: Friedreich’s ataxia (FRDA) is an inherited recessive neurodegenerative disorder caused by a homozygous guanine-adenine-adenine (GAA) repeat expansion within intron 1 of the FXN gene, which encodes the essential mitochondrial protein frataxin. There is still no effective therapy for FRD...

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Detalles Bibliográficos
Autores principales: Kalef-Ezra, Ester, Edzeamey, Fred Jonathan, Valle, Adamo, Khonsari, Hassan, Kleine, Paula, Oggianu, Carlo, Al-Mahdawi, Sahar, Pook, Mark A., Anjomani Virmouni, Sara
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9909538/
https://www.ncbi.nlm.nih.gov/pubmed/36777637
http://dx.doi.org/10.3389/fnins.2023.930422