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Excision of the expanded GAA repeats corrects cardiomyopathy phenotypes of iPSC-derived Friedreich’s ataxia cardiomyocytes.

Friedreich’s ataxia is caused by large homozygous, intronic expansions of GAA repeats in the frataxin (FXN) gene, resulting in severe downregulation of its expression. Pathogenic repeats are located in intron one, hence patients express unaffected FXN protein, albeit in low quantities. Although FRDA...

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Bibliografiske detaljer
Udgivet i:Stem Cell Res
Main Authors: Li, Jixue, Rozwadowska, Natalia, Clark, Amanda, Fil, Daniel, Napierala, Jill S., Napierala, Marek
Format: Artigo
Sprog:Inglês
Udgivet: 2019
Fag:
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC6853280/
https://ncbi.nlm.nih.gov/pubmed/31446150
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.scr.2019.101529
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