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Article

Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia.

Nature chemical biology - 1 Oct 2006

Herman David, Jenssen Kai, Burnett Ryan, Soragni Elisabetta, Perlman Susan L, Gottesfeld Joel M

Abstract excerpt

Expansion of GAA x TTC triplets within an intron in FXN (the gene encoding frataxin) leads to transcription silencing, forming the molecular basis for the neurodegenerative disease Friedreich's ataxia. Gene silencing at expanded FXN alleles is accompanied by hypoacetylation of histones H3 and H4 and trimethylation of histone H3 at Lys9, observations that are consistent with a heterochromatin-mediated repression...

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Source

Identifiers

PMID: 16921367
DOI: 10.1038/nchembio815

Genes

FXN
GAA

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia.PMID 16921367Read ↗

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