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

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

IntroductionFriedreich’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 FRDA,...

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Bibliographic Details
Main Authors: Ester Kalef-Ezra, Fred Jonathan Edzeamey, Adamo Valle, Hassan Khonsari, Paula Kleine, Carlo Oggianu, Sahar Al-Mahdawi, Mark A. Pook, Sara Anjomani Virmouni
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-01-01
Series:Frontiers in Neuroscience
Subjects:
Friedreich’s ataxia
FRDA
FXN
frataxin
GAA repeat
mouse model
Online Access:https://www.frontiersin.org/articles/10.3389/fnins.2023.930422/full

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https://www.frontiersin.org/articles/10.3389/fnins.2023.930422/full

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