The mismatch repair system protects against intergenerational GAA repeat instability in a Friedreich ataxia mouse model

Friedreich ataxia (FRDA) is an autosomal recessive neurodegenerative disorder caused by a dynamic GAA repeat expansion mutation within intron 1 of the FXN gene. Studies of mouse models for other trinucleotide repeat (TNR) disorders have revealed an important role of mismatch repair (MMR) proteins in...

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Main Authors: Ezzatizadeh, Vahid, Pinto, Ricardo Mouro, Sandi, Chiranjeevi, Sandi, Madhavi, Al-Mahdawi, Sahar, te Riele, Hein, Pook, Mark A.
Formato: Artigo
Idioma:Inglês
Publicado em: 2012
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3556645/
https://ncbi.nlm.nih.gov/pubmed/22289650
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.nbd.2012.01.002
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