Transcriptional profiling of isogenic Friedreich ataxia neurons and effect of an HDAC inhibitor on disease signatures

Friedreich ataxia (FRDA) is a neurodegenerative disorder caused by transcriptional silencing of the frataxin (FXN) gene, resulting in loss of the essential mitochondrial protein frataxin. Based on the knowledge that a GAA·TTC repeat expansion in the first intron of FXN induces heterochromatin, we pr...

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Wedi'i Gadw mewn:
Manylion Llyfryddiaeth
Cyhoeddwyd yn:J Biol Chem
Prif Awduron: Lai, Jiun-I, Nachun, Daniel, Petrosyan, Lina, Throesch, Benjamin, Campau, Erica, Gao, Fuying, Baldwin, Kristin K., Coppola, Giovanni, Gottesfeld, Joel M., Soragni, Elisabetta
Fformat: Artigo
Iaith:Inglês
Cyhoeddwyd: American Society for Biochemistry and Molecular Biology 2019
Pynciau:
Molecular Bases of Disease
Mynediad Ar-lein:https://ncbi.nlm.nih.gov/pmc/articles/PMC6369281/
https://ncbi.nlm.nih.gov/pubmed/30552117
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.RA118.006515
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