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Article

SIRT1 inhibition alleviates gene silencing in Fragile X mental retardation syndrome.

PLoS genetics - 7 Mar 2008

Biacsi Rea, Kumari Daman, Usdin Karen

Abstract excerpt

Expansion of the CGG.CCG-repeat tract in the 5' UTR of the FMR1 gene to >200 repeats leads to heterochromatinization of the promoter and gene silencing. This results in Fragile X syndrome (FXS), the most common heritable form of mental retardation. The mechanism of gene silencing is unknown. We report here that a Class III histone deacetylase, SIRT1, plays an important role in this silencing process and show that...

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Source

Open in PubMed Open full text

Identifiers

PMID: 18369442
DOI: 10.1371/journal.pgen.1000017
PMCID: PMC2265469

Genes

FMR1
SIRT1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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SIRT1 inhibition alleviates gene silencing in Fragile X mental retardation syndrome.PMID 18369442Read ↗

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Rescue of Fragile X Syndrome Neurons by DNA Methylation Editing of the FMR1 Gene.Semantic proximity Read ↗

The distribution of repressive histone modifications on silenced FMR1 alleles provides clues to the mechanism of gene silencing in fragile X syndrome.Semantic proximity Read ↗

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