Cargando…
Selected Histone Deacetylase Inhibitors Reverse the Frataxin Transcriptional Defect in a Novel Friedreich’s Ataxia Induced Pluripotent Stem Cell-Derived Neuronal Reporter System
Friedreich’s ataxia (FRDA) is a neurodegenerative disorder caused by the expansion of guanine–adenine–adenine repeats within the first intron of the frataxin (FXN) gene. The location and nature of the expansion have been proven to contribute to transcriptional repression of FXN by decreasing the rat...
Autores principales: | Schreiber, Anna M., Li, Yanjie, Chen, Yi-Hsien, Napierala, Jill S., Napierala, Marek |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2022
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8904878/ https://www.ncbi.nlm.nih.gov/pubmed/35281493 http://dx.doi.org/10.3389/fnins.2022.836476 |
Ejemplares similares
-
Selected missense mutations impair frataxin processing in Friedreich ataxia
por: Clark, Elisia, et al.
Publicado: (2017) -
Premature transcription termination at the expanded GAA repeats and aberrant alternative polyadenylation contributes to the Frataxin transcriptional deficit in Friedreich’s ataxia
por: Li, Yanjie, et al.
Publicado: (2022) -
Friedreich's ataxia – a case of aberrant transcription termination?
por: Butler, Jill Sergesketter, et al.
Publicado: (2015) -
Somatic instability of the expanded GAA repeats in Friedreich’s ataxia
por: Long, Ashlee, et al.
Publicado: (2017) -
Mitochondrial damage and senescence phenotype of cells derived from a novel frataxin G127V point mutation mouse model of Friedreich's ataxia
por: Fil, Daniel, et al.
Publicado: (2020)