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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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| Главные авторы: | , , , , , , |
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| Формат: | Artigo |
| Язык: | Inglês |
| Опубликовано: |
2012
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| Предметы: | |
| Online-ссылка: | 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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